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Bone Tumors
Mohamed Sobhy
Quick Guide
Bone Tumors
Index
Table of Contents
Tumor Principle...................................... 1-12
Myelogenic Tumors ............................... 13-28
Fibrous Tumors ..................................... 29-40
Osteogenic Tumors ............................... 41-62
Chondrogenic Tumors .......................... 63-75
Cystic Lesions ........................................ 77-93
Miscellaneous ........................................ 95-100
Soft Tissue Sarcomas ............................ 101-123
Tumour Principles
Staging Musculo-skeletal Neoplasms (Enneking)
Classification based on:
1. Histological grade (G)
2. Site (T)
3. Metastases (M)
Enneking staging system was tested retrospectively on 397 cases of bone & soft tissue tumours.
Shown that prognosis varied with the stage.
The system aids in assessing prognosis & planning management
NB: Applies to lesions of connective tissue, not primary lesions of round cell origin (eg
leukaemia's, lymphomas, myeloma or Ewings)
Enneking's Surgical Stages
STAGE GRADE
SITE
METASTASES
1A
1B
2A
2B
3
None(M0)
None(M0)
None(M0)
None(M0)
Yes(M1)
Low(G1)
Low(G1)
High(G2)
High(G2)
Low(G1) or
High(G2)
Intracompartmental(T1)
Extracompartmental(T2)
Intracompartmental(T1)
Extracompartmental(T2)
Intracompartmental(T1) or
Extracompartmental(T2)
Parosteal osteosarcoma
LG medullary osteosarcoma
Giant Cell Tumour
Secondary Chondrosarcoma
Fibrosarcoma
Myxoid Liposarcoma
HIGH (G2)
classic osteosarcoma
radiation osteosarcoma
Paget's osteosarcoma
Primary Chondrosarcoma
Malignant Fibrous Histiocytoma
Pleomorphic Liposarcoma
Neurofibrosarcoma (Shwannoma)
Rhabdomyosarcoma
Synovioma
Extracompartmental
intraosseous
intra-articular
Intrafascial compartments:
ray of hand or foot
posterior or anterior leg
ant, med, post thigh
buttocks
volar or dorsal forearm
anterior or posterior arm
pericapsular
Tumour Workup
1. Clinical examination (age, sex, site and past history)
o Thyroid
o Liver
o Breasts
o Kidney
o Chest
o Rectal (prostate & rectal tumours)
2. Bloods
o FBC (leukaemic cells etc)
o ESR (often elevated)
o Biochemistry (Ca++, PO4, liver enzymes and Alkaline Phosphatase) -> mets
o Acid Phosphatase (prostate and increased with metastatic deposits)
o Thyroid function tests
o PSA
o Serum Protein Electrophoresis (Myeloma)
3. Urinalysis
4. Urine Bence-Jones (myeloma)
5. CXR
6. Abdominal ultrasound
7. Bone scan other sites
8. MRI soft tissue extent and association with nerves and vessels
9. CT of lesion and chest ( staging)
10. Angiography tumour blood supply and relationship to major vessels
11. Biopsy
Diagnosis
1. AGE PREDILECTION
0 - 5yr
6-18yr
19-40y
40+yrs
Benign
Malignant
Other
1.
2.
1.
2.
1. Osteomyelitis
2. healing/ stress fracture
1. Osteomyelitis
2. Fibrous Dysplasia
3. Osteofibrous Dysplasia
laeukaemia
Metast.Neuroblastoma
Ewings Sarcoma
Osteosarcoma
1. Ewings Sarcoma
1. Metastases
2. MM & Lymphoma
3. osteo,chondro,fibrosarcoma
1. Hyperparathyroidism
2. Osteomyelitis
3. Paget's
2. NUMBER OF LESIONS
Is it monostotic or polyostotic?
3. TUMOR LOCATION
Metaphyseal Diaphyseal Epiphyseal
Medullary (central, eccentric) - Cortical Juxta-cortical as:
parosteal osteosarcoma - no space bet lesion & cortex
myositis ossificans - space between lesion & cortex
Exostoses (cartilage capped) - shares cortex bone
4. LYTIC, SCLEROTIC, MIXED
4
9. PERIOSTEAL REACTION:
1. Continuous:
1. Solid: osteoid osteoma
2. Linear: ABC, GCT
3. Lamellated: Onion peel
2. Interupted: Codmans U
3. Complex: Sun ray and Hair on end appearance
10. SOFT TISSUE MASS: usually denotes primary lesions
Site Predilection
Behavior Grading
Typical example
Classification
Benign/latent (self limiting)
Benign/active (grow capsule)
Benign/aggressive (no capsule)
Malignant/low-grade
Malignant/high-grade
Bone
Nonossifying fibroma
Aneurysmal bone cyst
Giant-cell tumor
Parosteal osteosarcoma
Classic osteosarcoma
Trabeculations
Lesion
Gian Cell Tumor
ABC
Chondromyxoid fibroma
Non ossifying fibroma
Hemangioma
Trabecular Pattern
Delicate and thing
Delicate & horizontal
Coarse and thick
Lobulated
Striated and radiating
Soft Tissue
Lipoma
Angiolipoma
Aggressive fibromatosis
Myxoid liposarcoma
MFH
RADIOLOGICAL FEATURES
Phemister's Law = commonest site of infection & tumours is ! fastest growing site of ! long bone
To see a lucent lesion in bone, an estimated 30 - 50 % of the bone must first be lost
Features of Malignancy:
Metaphyseal:
Expand bone:
Spinal Lesion:
1. size of lesion (small is
- adults metastases /
Simple (central)
Giant cell tumour
good, big is bad)
myeloma / hemangiomas
FCD (eccentric)
ABC
2. cortical destruction
- young:
Simple bone cyst
3. No sclerosis margin
body: histiocytosis
4. Soft tissue mass
arch: ABC /O.blastoma
osteoid osteoma.
-sacrum chordoma
osteoid osteoma
Enchondroma- speckled
Fibrous Dysplasia = a long
Giant cell tumours nearly
looks as Brodie's abscess
cyst, small bones
lesion in a long bone
always occur near a joint
Enchondroma, simple bone
surface (in mature bone)
cyst & fibrous dysplasia
can look very similar
Cartilage tumours - have speckled calcification
Lytic infection- mimics any
Histiocytosis X
Chondroblastoma= Giant cell tumour of children. Only tr
bone lesion.
(Eosinophilic granuloma)arising in the epiphysis
flat bones usually,
Chondromyxoid fibroma- older Pt., eccentric, speckled
commonest is skull
Hydatid- rural
Hyperparathyroidism- brown tumour, phalangeal erosions, sclerosis, subperiosteal bone resorption, osteitis fibrosa cystica
Certain bones in the body can be considered "epiphyseal equivalents" for purposes of differential diagnosis. These include
the patella, the calcaneus, and most apophyses. Therefore, for lucent lesions in these areas, one should include the classic
epiphyseal entities such as chondroblastoma (children), giant cell tumors (adults) and aneurysmal bone cysts.
Buzzwords:
Onion Peel
Punched-out lesion
Moth-eaten
Permeative pattern
Rat-bite lesions
Pencil in cup
Blow-out
Soap bubble
Nidus
Sunburst spiculation
Popcorn balls
Sled runner tracks
Bear bite
Smoke goes up a chimney
Gracile bones
Erlenmeyer flask
Scallops from without
Scallops from within
Ground glass
Salt & pepper skull
Stress lines
Looser's lines
Ewing Sarcoma
MM, Eosinophilic granuloma
Round cell tumours
Round cell tumours
Congenital syphilis
Aneurysmal bone cyst
Aneurysmal bone cyst
Giant cell tumour
Osteoid osteoma
Osteosarcoma
Cartilage tumours
Ollier's & Mafucci's
Fibrosarcoma
Medullary infarct
Osteogenesis imperfecta
Gaucher's disease
Neurofibromatosis
FD, NOF, SBC, enchondromas, chondromyxoid & chondrosarcoma
Fibrous dysplasia, enchondromas
Paget's disease
Paget's disease (on convex/ tension side of bone)
Rickets / osteomalacia (on concave/ compression side of bone)
MRI features:
most tumours are dark on T1 & bright on T2
Desmoids, scar tissue, fibrous tissue & cortical bone are dark on T1 & T2
Lipomas, liposarcomas & haemangiomas are bright on T1 & T2
6
Tumour
Ewings Sarcoma
MM
RCS
Hodgkin
Lymphosarcoma
EG (BFH)
MFH
Fibrosarcoma
Desmoid
Osteosarcoma
Osteoid Osteoma
Osteoblastoma
Osteochondroma
MHE
Enchondroma
Chondroblastoma
Chondromyxoid
Chondrosarcoma
Giant cell Tumor
ABC
Adamantinoma
Chordoma
AGE
nd
2 decade
5th & 6th decade
5th & 6th decade
20-30
40-60
< 30y
10-30
10-30
<20y
3-12y
10-50
10-20
10-30
30-60
10-40
40-70
Origin
Endothelial elements of BM PNET
Plasma cells
BM lymphoid cells
Lymphoid reticulum
Lymphocytes
Histiocytes
Fibroblasts
Fibroblasts
Reaction to add.magnus stress
Bone forming mesenchymal cells
Unknown Reactive bone lesion
Unknown
Physeal herniation
AD & EXT 1&2 genes
chondroblasts
chondroblasts youngs GCT image
Physeal remnants
Cartilage forming cells
Supporting cells of bone CT
Aneurysmal malformation
Epithelial origin
Remnants of notochord
Rank
3rd common
1ry malign.BM
2:1
2:1
3:1
Rare
2nd Common
Commonest Benign
2:1
2:1
2:1
Extremely rare
Rare
Eccentric metaphyseal
4th common sarcoma
chrornosomal abnormalities
Radiological Investigations
Bone Scan
Tc99m-HDP (Diphosphonates labelled Technetium 99) usually used in dose of 500 - 600 mBq
Osteoid osteoma -> increased activity in blood pool phase as well as delayed bony phase
Inflammatory lesions also increased activity in blood pool scan
Gallium 67 has also been utilised
CT Scans
Indicates extent of bony involvement / destruction
Good for detecting subtle cortical disruption, fracture, calcification or ossification
Not as good as MRI for soft tissue extent. Can detect soft tissue masses >/= 5mm diam.
Useful to stage - eg. lung secondaries
Angiography
Feeding vessels identified as well as the tumour proximity to major vessels
Displacement of vessels by tumour -> access for excision of the tumour
Embolisation of vascular tumour prior to surgery
Intra-arterial chemotherapy
MRI
Good soft tissue definition (better than CT) and extra-osseous extension, joint involvement, skip
lesions and epiphysial extension -> staging -> extent of tumour both intra and extra medullary
Able to image in any plane - thus good for pelvic/sacral lesions
T1 images best for looking at extent of BM involvement & T2 useful for evaluating cortical
bone and soft tissue extent (NB - CT better for showing areas of calcification / ossification)
Best technique to identify haemorrhage/oedema/inflammation- eg prior biopsy
Oedema usually surrounds malignant lesions & is unusual around benign tumours
10
SURGICAL
PROCEDURES
(Aim:
remove the lesion minimal risk of local
recurrence)
Limb Salvage
Criteria for limb salvage:
1. local control of the lesion must
be at least equal to amputation
2. the saved limb must be
functional
Types of osseous resection
1. Inter calary (between joints)
2. Intra articular (one side of joint)
3. Extra articular (both sides of the
joint)
Various methods of salvage eg,
endoprosthesis, allograft, composite,
arthrodesis
Relative contraindications: Pathological
# & Skeletal immaturity
metastasis
Distal
is
not
a
contraindication
Choose anatomical location favours
reconstruction & allows wide margins
Surgical Margins:
1. Intra-lesional
o through the tumour & leaves
macroscopic tumour
o not therapeutic
2. Marginal
o Via
pseudo-capsule/reactive
zone (contains inflame.cells,
oedema, satellites of tr)
o controls non-invasive benign tr
o recurrence of malignant tumours
= 25-50%
3. Wide
o around reactive zone, leaving a
cuff of normal tissue
o skip lesions left
o recurrence of malig tr < 10%
4. Radical
o removal of entire compartment
or compartments
o distant metastases left
5. Amputation
o should be thought of as a form of reconstruction when function outweighs preservation
11
ADJUVANT CHEMOTHERAPY
Nomenclature:
"Adjuvant" = chemotherapy given to ttt micrometastases
"Neoadjuvant" = preoperative chemotherapy before resection of 1ry tumor.
Neo-adjuvant chemotherapy
= Staging Chemotherapy Restaging Surgery Trr kill rate Further treatment
(change chemo or introduce radiotherapy as indicated)
Enables action of agents against tumour to commence immediately
Efficacy of chemotherapeutic agents evident at the time of tumour resection
Reduces the mass and vascularity of the tumour prior to definitive surgery
Enables time for operative planning
Want 90% kill rate and if < 90% change agents
No y survival evident with different agents in those who do not respond to the initial
treatment. (? just identifies those patients with a good prognosis).
Commence adjuvant treatment once the wound has healed
May persist for 2 months to 2 years depending on the response
Localised disease = 60-70% long-term disease-free survival
May lead to:
1. Stunting of growth (catch up later)
2. Osteoporosis
3. AVN
4. Cisplatinum nephro & ototoxicity
5. Adriamycin cardiotoxicity
6. Vincristine Neurotoxicity
7. Chemotherapeutic induced malignancy usually blood forming eg leukaemias but also
may Ca bladder or skin (particularly cyclophosphamide)
Methotrexate
Binds to dihydrofolate reductase and therefore blocks purine synthesis.
Potentiates the effects of radiotherapy
Citravorum factor "rescue" replenishes the folate pool
Adriamycin
Cytotoxic antibiotic
Also potentiates radiotherapy
12
RADIOTHERAPY
Pre-operative radiotherapy may z size of the tumour, decreased chance of seeding at the time
of resection or if seeding z chance of viability of the shed cells
Effect of radiation
Direct = absorption by complex molecules causes rupture of chemical bonds with
damaging effects
Indirect = by ionisation & formation of highly reactive intracellular free radicals DNA
changes stop cell reproduction &z cell turnover loss of specific cell function
The effect on cancer cells not more rapid growth of cells but in the capacity for
recovery and repair of normal tissues compared poor capacity of cancer tissues
Destruction of small blood vessels on which growth of a tumour depends also
contributes to the effect and induction of an inflammatory response may -> destruction of
cells weakened by radiation
Increased sensitivity of cells in the presence of oxygen up to a critical level but above this
level (about that of normal atmospheric pressure) sensitivity does not increase
appreciably (hypoxic tissue has decreased radio sensitivity)
Radiosensitivity of a tissue is directly proportional to its mitotic activity and
inversely proportional to the degree of differentiation of its cells
Adverse effects:
1. Joint -> stiffness and loss of function -> physio
2. Subcutaneous fibrosis
3. Children -> premature fusion of growth plates
4. Irradiation induced sarcoma
5. Enteritis, diarrhoea, obstruction and bleeding
6. Cystitis and hepatitis
7. Scoliosis may develop therefore include both sides of vertebrae
8. Muscle atrophy and fibrosis
9. Erythema of skin and hyper pigmentation
10. Hair loss and skin flaking
11. Lymphoedema -> need to screen a strip of skin -> lymphatic drainage
Defiinitions
Rad (radiation absorbed dose) is a measure of the energy imparted to the matter by
ionising radiation per unit mass (1 Rad = 100 erg/gram (0.01j/kg)
Grays (Gr) = 1 joule of energy absorbed by a mass of 1kg (equivalent to 100rad)
13
`xzx|v g
o
o
o
o
o
o
o
Ewing's Sarcoma
Multiple Myeloma
Reticulum Cell Sarcoma
Hodgkin Lymphoma
Lymphosarcoma
Chloroma
Leukemia
15
Ewings Tumour
It is a primitive neuroectodermal tumour. It is the 3rd most common malignant 1ry bone tumor
(after MM & Osteosarcoma). Usually it occurs < 30 y, rapidly destroy the cortex and reaches the
soft tissues & metastasize. Usually the tumor is associated with fever and constitutional symptoms
Origin:
Incidence
Pathology
Site:
Long bones: FEMUR, tibia, fibula, metatarsals, humerus,
radius, ulna, metacarpals
o Flat bones: Scapula, pelvis, ribs, clavicle, & skull
o Irregular bones: Tarsus esp. The calcaneus & Vertebrae
Localization: DIAPHYSEAL
Macroscopically:
o Grey like BRAIN MATTER DD inspisated pus of OM /or (red
currant jelly if hgic)
o Occupy the medulla of the bone
o Growth usually exceeds nutrition supplies tissue lysis; hence the fever & the
Cysts filled necrotic debris (pus like) & the lytic appearance in PXR
o Necrotic lamellae in relation to the tumor is common feature
o Tumor extend via Haversian system to the surface leading to:
NBF in episodes ONION PEEL
Poorly demarcated and soft tissue extension common
Microscopically:
o HIGHLY CELLULAR tumor made of packed solid sheets of small cells
o cells are quite UNDIFFERENTIATED
o MONOTONOUSLY HOMOGENOUS with that conform to one shape round, or polyhedral
o Minimal intercellular substance
o NUCLEI: large / oval / HYPERCHROMATIC / fine regular chromatin / mitotic figures
o A ring of 7-8 cells around a central area of necrosis= "PSEUDOROSETTE" may form
o Within the centre of this rosette fibrils may be seen (=true rosette ccc of neuroblastoma)
o Hemorrhage and necrosis are typically present
o +ve PAS, SSS (sliver stains are of no help as it stains both collagen & reticulen fr)
o -ve reticulin stain
Spread:
o To distant sites via BLOOD and LYMPHATICS LN (? MULTI-CENTRIC from the onset)
o To other bones
o
16
Clinically
1.
2.
3.
4.
5.
60% occur in long tubular bones (also pelvis ribs and scapula)
Considered a systemic disease
PAIN: ..........................................................................90%
o Vague, throbbing
o Starts intermittent long periods of remission (unlike any malignancy pain)
o Eventually, it becomes constant and severe
o worse at night
TENDERNESS of the lesion area
o spread of tumor via Haversian canals to outside to involve the soft tissues
including the nerves marked mass, all this before any PXR finding
o when Haversian vv are occluded sclerosis & lysis according to whether the vv
are completely or incompletely occluded
LUMP:
o Painful
o Hard
o Warm
o Diaphyseal
o Tender
o Smooth surface
o Ill-defined edges o Fusiform
o Slowly growing and its size y in episodes
METASTATIC deposits at presentation .........................(30%) Lung & LN most common
Patient may be ill and sometimes PYREXIAL ...............(20%)
o Fever may lead to mistaken diagnosis of osteomyelitis
o necrosis of the tissues; whose cells are closely related to pus cells
o Associated with y WBC, ESR
Pathological FRACTURE is rare ...................................(15%)
NEUROLOGICAL symptoms: .........................................(55% of pt spinal lesion)
6.
7.
8. CACHEXIA
Serology
Anemia
y ESR & WBC
y serum Alkaline Phosphatase
Vanyl Mandilic Acid (VMA) in urine to exclude neuroblastoma
Glycogen stains (PAS) to exclude Reticulum Cell Sarcoma
Electron Microscopy usually differentiate RCS
Biopsy is necessary
Cyto-genetics to determine the cell origin & translocation
PNET primitive neuro-ectodermal tumor t(11:22)(q24:q12)
Specific surface glycoprotein HBA71 & MIC2 are specific
X-Rays
it is permeated by the tumor to produce another layer. Other periosteal reactions are also
present i.e. SUN RAY or CODMANS U
Rarefied area in medulla, but bone marrow infiltration is often not obvious on plain x-ray
Often the cortex is perforated
May look like osteomyelitis but diaphyseal
17
MRI
Bone Scan
increased uptake
Differential Diagnosis:
1.
2.
3.
4.
5.
6.
7.
Treatment
Prognosis
14% of long term survivors develop secondary tumours and 1 - 2% are malignant (eg
leukaemia or osteosarcoma)
Young males and pelvic lesions -> worse prognosis.
If less than 10% viable tumour after chemotherapy =80% cure ;If not =20-30% cure
18
Multiple Myeloma
Definition:
MM is a malignant tumor of PLASMA CELLS that causes widespread osteolytic bone damage
=plasma cell myeloma = plasmacytic myeloma = plasmacytoma = myelomatosis
Cytogenetic: recently common ch.abnormality at IG HEAVY CHAIN SWITCH REGION was found
tiology theoretical
Chronic inflammatory diseases
Herpes Virus 8 (HPV8)
PDF: Asbestos, Benzene, Pesticides, Radiation of atomic bomb
Epidemiology:
Commonest 1ry malignancy of bone > 40y (~ 40% of bone 10% of blood 1% of cancer)
May affect any bone with haematopoietic red marrow (spine, skull, ribs, sternum and pelvis)
Age 40-60 year
:=2:1
BLACK:white people also 2:1
Pathogenesis:
1.
2.
3.
4.
5.
Plasma cells are the most mature form of B-lymphocytes i.e. they secrete mature IgGs
Thats why it is ccc by OVERPRODUCTION OF MONOCLONAL IgG, IgA, and/or light chains
Sequelae depend on the site involved:
Skeletal:
MM produce OSTEOCLASTIC ACTIVATING FACTOR
bone is destroyed and replaced by tr pain & spinal cord compression & path#
hematologic
BM infitraltion by tr neutopenia, anemia, thrombocytopenia (PANCYTOPENIA)
Myeloid components interact clotting factors defective aggregation bleeding
Renal
Renal infiltration by plasma cells myeloma, light chain nephropathy, amyloidosis,
GLUMERULOSCLEROSIS hypercalcemia & hypercalcuria
Nervous:
Amyloid infiltration of nerves radiculopathy, cord compression, skeletal destruction
General
HYPER VISCOSITY sludging of the myeloid infiltrates in capillaries
Purpura, retinal hge, papilledema, coronary ischemia, CNS confusion or vertigo
Pathology:
Site:
FLAT BONES are the commonest (site of red bone marrow) skull/spine/sternum/ scapula/ribs
19
Histology
Biopsy reveals SHEETS of densely packed plasma cells
ROUND or OVOID cells
1. ECCENTRIC NUCLEI typical CART WHEEL appearance = CLOCK FACE nuclei
2. CLUMPED NUCLEAR CHROMATIN beneath the nuclear membrane
3. BASOPHILIC CYTOPLASM ccc clear area HALO
4. The degree of cytological atypia of these cells has no prognostic value
OSTEOLYSIS occurs y cytokines released by the plasma cells osteoclastic resorption
HISTOLOGICAL CLASSIFICATION of MM:
1. Plasma myeloma
plasma cells
2. Myelocytoma
myeloblasts
3. erythroblastoma
Erythroblast
4. lymphocytoma
lympoblasts
Types:
1. Multiple myeloma
2. Solitary myeloma
3. Diffuse myeloma (myelomatosis)
4. chloroma: special type occurs in the skull; its cut section shows green color pigment
production that rapidly fades
Presentations
1- Due to Mass:
1. PAIN
2.
3.
4.
5.
6.
7.
insidious bone pain starts in back, thorax, pelvis & precedes PXR findings
Duration of symptoms before dx ~ 9 mo
lytic effect on bone: Cortex is eroded from inside & Cancellous bone is melted away
Pathological FRACTURES
The tumor may remain silent till this event
thining and weakness of cortical bone
NEUROLOGICAL manifestation:
Compression fracture of a vertebra nerve root injury
Sciatica is a common & sometimes initial symptom
ANEMIA: extensive BM replacement
BLEEDING tendency: thrombocytopenia + interaction clotting factors by m proteins
CONSTITUTIONAL symptoms related to anaemia, thombocytopenia and renal failure
HYPERCALCEMIA: nausea, fatigue, thirst
1.
2.
3.
4.
5.
20
Investigations
CBC:
Pancytopenia (normocytic normochromic anemia)
Bleeding tendency (thrombocytopenia-abnormal coagulation)
y ESR (>100 mm/hr)
y Reticulocytes & Rouleaux formation
Chemistry:
o y Ca, AlkPh
o y creatinine
o y s.uric
o y LDH & 2microglobulins
o y serum proteins: >10gm/dl reversed A/G ratio (mainly in the globulin fraction)
S.Protein Electro-Phoresis (SPEP)& high resolution SPEP
o N/z alb
o N 1 & 2 &
o y Globulin = MONOCLONAL GAMMOPATHY (M-COMPONENT > 30G/L is mandatory to Dx)
o Accumulation of light chain proteins found in 20% and execreted in urine BJP
Immune fixation to determine the type of Ig:
60% IgG
20% IgA
1% IgD (IgM & IgE are rare)
Urine:
o y ca
o z sp.gravity (RF)
o Amyloid cast
o
o
o
o
C precipitate 100
Radiology
PXR Skeletal survey is the MOST SENSITIVE; as Tc scan fail to show y uptake in 25%
The characteristic appearance of MM:
o Multiple, round, lytic, lesions
o Lesions are PUNCHED OUT, well defined, variable in size
o
o
NO PERIOSTEAL REACTION
Generalized demineralization
The characteristic appearance of single plasmacytoma:
o BUBBLY expansion of a single bone (rib or spine posterior arch)
o Associated SOFT TISSUE MASS
MRI
is useful for delineating spinal lesions & early cord compression
low T1 & high T2 (related to muscle); and it is enhanced the use of gadolinium
CT: Usually not needed in most of the patients; but it shows the extent of bony affection
Tc99m: COLD SPOTS (lesions are osteoclastic and not osteoblastic)
21
Treatment
Radiotherapy
o MM is Radio & chemosensitive, and reossification of tr defects may occur in months
o Radiotherapy is recommended for intractable bone pain & <50% affection of bone
o Initially there might be additional bone loss inflammation NWB for 4-6 weeks
Drugs: although MM is incurable many drugs were proposed to have a good effect
o Interferon 2b + prednisolone prolong disease free interval
o Bisphosphonates: z pain, recurrence, hypercalcaemia.
Chemotherapy
o Melphalan/Prednisone (MP) is the standard ttt for MM since 60s
o MP 4days 4wks MP these courses are repeated for a yearPalliative only
o Recent combination Vincristine, Adriamycin, Dexamthazone (VAD) especially for relapse
o Response to ttt & tumor burden are measured by M-component in serum or urine
(50% reduction is a good response)
Surgery
o Prophylactic IM nails for femoral, humeral deposits
o ORIF of other pathological fractures IM nails MMA
o spinal decompression for root compression or fracture
o if joint is affected arthroplasty is thought about
Prognosis
distribution
common sites
predominant pattern
Morphology
MM
Symmetric
Skull, mandible, spine body (arch)
Osteolytic
Will defines
uniform
Secondaries
Asymmetrical
These sites are rare
Osteolytic or sclerotic
Ill defined
Variable sizes
22
Bone Lymphoma
(Non-Hodgkin's) reticulum cell sarcoma
It is the 1ry malignancy of BM LYMPHOID CELLS. Although, 1ry lymphoma is potentially curable & 2ries
are fatal, both have the same cell type; but they are completely different in behavior & also in ttt.
Disseminated reticulum cell sarcoma is a generalized disease affecting bone & other lymphoid tissues
Criteria for diagnosis (definition):
HISTOLOGICALLY PROVED to be Lymphoma
NO OTHER Lymphoid affection
Solitary or multi bone lesion REGIONAL LN
tiology
Epidemiology
Pathology:
Sites:
Long bones: FEMUR / humerus / tibia / fibula / radius / ulna
Flat bones: scapula (acromion) / ILIUM
Irregular bones: patella / VERTEBRAE / tarsal bones
Localization:
METAPHYSIO-DIAPHYSEAL lesion
Take at least the shaft of the bone
Macroscopic
NOTHING CHARACTERISTIC; PINKISH GREY mass
Begins IM and erodes bone from in to extend to soft tissues
Sometimes erosion is slow giving the chance of periosteum to
lay down shell of bone cortical expansion
Microscopic:
LAR
ARGE PLEOMORPHIC cells ( small uniform cells in Ewing) arranged
in SYNCYTIAL bands that are supported by primitive reticulum
Cell membrane is usually ILL DEFINED or IRREGULAR PSEUDOPODS
Cytoplasm:
o smooth / granular / basophilic
o cytoplasm : nuclear volume > 1:1
Nucleus:
o LAR
ARGE / HYPERCHROMATIC / REN
ENIFORM in mature tumor forms
o frankly lobulated in younger cells (may appear as a cluster of nuclei)
o High MITOTIC figures (> Ewing)
o COARSE IRR
RREGULAR CHR
HROMATIN (may look like Ewing=fine and regular)
o Nucleoli are evident
No intracellular glycogen ( Ewing)
+ve Argenophilia (SSS) reticulin fibrils (in Ewing it is +ve fine collagen fibrils)
23
Metastasis:
Late metastasis unlike Ewing
Mainly metastasize to LN > bone the opposite of the situation in Ewing
Both kill by lung metastasis
Staging:
SI
one bone lesion soft tissue mass
SII
Two lesions of the same side of the diaphragm
Or One lesion + regional LN
SIII
Two lesions on both sides of the diaphragm
SIV
BM or CNS affection
Clinically (slower than Ewing)
40-60
X-Rays
1. DOES NOT AFFECT LESS THAN OF THE BONE (extensive erosion). PXR often underestimate the
Tumor behavior:
Lytic: Moth-eaten or Permeative ... 65%
Mixed: ............................................ 30%
Pure sclerotic: ................................ 5% (may be more common Ewing)
PATCHY MATRIX OPACIFICATIONS reactive NBF (indicate slow growth)
MOTTLED APPEARANCE: alternating areas of normal bone, lytic tumor & NBF
Little periosteal reaction y when cortex is eroded
3. SOFT TISSUE MASS:
Ewings
Osteosarcoma
2ry
MM
MFH or fibrosarcoma: arise from the fibrous elements of the endosteum,
mainly IM extension, and saucerize the cortex late
24
Difference
1- Incidence:
2- Age:
3- Pathology:
A/ Site:
B/ Macro. :
Ewings sarcoma
More common
5-15
Diaphyseal
Like brain matter, cheese or inspisated
pus (always mistaken osteomyelitis)
Necrotic lamellae
Medium size
Uniform
Contain glycogen
1 : 1 or 1 : 2
z
Rosette arrang. & in center by sss,
collagen fibrils are demonstrated
Large
Pleomorphic
No glycogen
y
y
Reticulin fibrils by special silver stain (sss)
BAD
severe
z
Much periosteal reaction
some bone destruction
Onion Peel
Most lethal bone sarcoma (95% )
It arises from BM very rapidly.
Metastasize early by all means
May be Radiosensitive but hazardous
to children
GOOD
Less severe
yy
Little periosteal react. But destructive &
expanding lesion
Mottled patchy matrix
30% can be cured by radio early Dx
It grows more slowly, metastasizes later
Excellent results even after LN metastasis
Radiosensitive more than other BM tumours
Cl Micro.:
1) Cells
Investigations
25
Treatment (mainly medical)
1.
2.
3.
4.
5.
Prognosis
Lymphoma of bone has the best prognosis of all primary malignant bone tumors
30% cure correct early Dx
60% 5ysr ttt
Excellent results even after LN metastasis
It grows more slowly, metastasizes later
Radiosensitive more than other BM tumours
Other Lymphomas
Hodgkin
Lymphosarcoma
Follicular lymphoma
Lymphatic Leukemia
These tumors arise from
LN
Spleen
Intestinal Follicles
Liver
Skin
Thymus
In that order, and affect the BM reticulum as a part of generalized lymphoma, or may be 1ry
Hodgkin
26
20-30 y
1. LN:
The earliest manifestation
Discrete, smooth, soft rubbery
Painless, mobile
Late they become hard
2. CONSTITUTIONAL symptoms:
BEL EBSTEIN INTERMITTENT FEVER 15 d on & 15 d off
Anemia
z wt
3. ABDOMENAL examination
HSM LN
Ascites
4. BONY affection:
Late to occur after the disease is manifested in other tissues
Dull aching pain & tenderness
Cord compression
PXR
1.
2.
3.
4.
Lumbar spine:
Pelvis & Skull:
Ribs:
Long bones:
27
Investigations:
1. CBC:
a. Anemia
b. Leucocytosis or leucopenia + relative lymphocytosis
2. CXR:
a. Hilar LN
b. Pulmonary Hodgkins
3. Biopsy: is the main parameter for accurate diagnosis
Treatment
Lymphosarcoma
VERY RARE tumor of bone characterized by multiple bone affection if occur
Pathology:
Site: VERTEBRAE
Extend diffusely in the medulla ERODE THE CORTEX
Extend via Haversian canals subperiosteally to infiltrate soft tissues
NO NBF
Microscopic:
Clinically:
PXR:
Early PAIN
Vertebrae are commonly affected referred chest pain & LL pain
Pathological FRACTURE
Lytic PERMEATIVE lesions & NO NBF
Vertebral wedging
Treatment:
IRRADIATION z pain
Death in 3y
chloroma
It is a form of lymphatic or myeloid leukemia in tissues contain GREEN PIGMENT appear in cut sections
Children NEAR PUBERTY are the commonly affected
Rapidly FATAL in 5mo
Affects LN, spleen & bones; skull invade its structures esp.orbit exophthalmos & lid edema
Bones red BM also invaded by the tumor may extend to involve surrounding muscles &
tendons
Clinically, PXR, Microscopically: are the same as leukemia but:
Large ATYPICAL MONOCYTES
Mitotic figures
PRIMITIVE MYELOBLASTS
Hyperchromatic nuclei
Treatment: DXT
28
Leumkemia
Is a condition characterized by malignant production of immature lymphocytes (lymphoblastic) or
polymorphs (myeloblastic); resultant replacement of hemopoietic & lymphoid tissues
It occurs as acute or chronic forms ; the acute being common & predominant in children
Clinically:
1)
2)
3)
4)
5)
6)
7)
Fever
Anemia
z wt
HSM LN
Bone affection (50%): PAIN & palpable MASSES in superficial bones (e.g. Ulna)
Painful joints (synovial involvement)
Rib pathological FRACTURE
Pathologically:
CBC:
Anemia
Thrombocytopenia
Lymphopenia or lymphocytosis
Immature WBC
PXR
1. DIFFUSE OSTEOPOROSIS
2.
3.
4.
5.
Treatment:
IRRADIATION is effective for pain relief (eventually loses its effect)
MULTIAGENT CHEMOTHERAPY is the main ttt
Prognosis
Y|u g
o
o
o
o
o
o
Fibrous Dysplasia
Fibrous cortical defect & Non ossifying fibroma
Fibrosarcoma
MFH
BFH (Eosinophilic Granuloma)
Desmoplastic Fibroma
31
Fibrous Dysplasia
Non hereditary developmental disorder of bone forming mesenchyme a defective
osteoblastic differentiation & maturation; resultant abnormal growth. Unknown tiology
Incidence:
Genetics:
Pathogenesis
Pathology:
NEA:
Microscopic:
Types:
1.
2.
3.
4.
5.
32
Monostotic:
70%
Sites: Rib (28%), Femur (23%), Tibia, Craniofacial (10-25%), Humerus
CP:
Almost 10-30 y
Pain
Pathological #
Less severe deformity than polyostotic
Polyostotic:
30%
Sites: Femur (90%), Tibia (80%), Pelvis (70%), Ribs (60%), Skull (50%), UL,
Lumbar spine, Clavicle, Cervical spine
May be Monomelic, Unilateral, Bilateral
CP:
Pain, limp, Pathological #, usually before age of 10
Deformity and LLD
Femoral coxa vara + upper femoral bowing Shepherds Crook Deformity
Craniofacial:
Cherubism:
AD
Regress after adolescence
Broad protruded jaws; both maxilla & mandible are symmetrically affected
McCune Albright $
Polyostotic
Precocious Puberty
Caf au-lait spots
Clinically:
Wide spectrum; from monostotic asymptomatic cases to polyostotic markedly deformed pts.
70% of patients presents pain
Deformities by the age of 10y
Pathological fractures
Special presentation: according to type; e.g. blindness, hearing lossetc
PXR
Long bones:
Metaphyseal or diaphyseal lucent lesions (may extend to epiphysis after closure)
Lesions matrix is homogenous and classically have the Ground Glass appearance
Endosteal scalloping Bone expansion
Femoral coxa vara + upper femoral bowing Shepherds Crook Deformity
Bone may show calcifications & endochondral NBF, but absent periosteal reaction
Premature closure of physis may occur adult dwarfism
33
DDx
C o m p l i c a t io n s :
1. Malignant transformation:
0.4-1%
May take several years to develop
Commonest are: skull & facial lesions in monostotic / femur & facial in polyostotic
Osteosarcoma, fibrosarcoma are ! commonest transformation (chondrosarcoma is less)
PXR:
Rapid y in size
Change of the sclerotic lesion to be lytic
Clinically:
y pain
y size of soft tissue mass
2. Metabolic changes:
Hypophosphatemic rickets and osteomalacia occur FD
May be because FD secretes phophaturic hormone
T r e a t m e n t:
34
Clinically
X-Rays
Histology:
Differential Diagnosis:
1.
2.
3.
4.
5.
Treatment
Most spontaneously resolve or move to the diaphysis of the bone with growth
Pathological fractures:
usually heal with a normal amount of callus, but resolution of the fibroma may or may not
occur
intracapsular curettage is usually sufficient to promote healing of lesion, however, the
defect may be supplemented with bone grafts +/- stabilisation
Jaffe-Campanacci syndrome:
multiple FCDs
cafe-au-lait spots
mental retardation
hypogonadism
ocular & cardiovascular abnormalities.
35
Fibrosarcoma
Rare tumor that may arise in the medulla (central), or on surface
(periosteal)
Differentiation is difficult bet. LG fibrosarcoma & the aggressive
benign tumors (desmoplastic fibroma & periosteal desmoid)
Also the diff. bet. HG fibrosarcoma and osteosarcoma & MFH
Incidence
2% primary malignant bone tumours
Middle age
But it occurs in any age and sex
tiolo
gy
og
no definite cause
Mutations and translocations are suspected
2ry fibrosarcomas (on top of other pathologies):
VonRecklinghausen $
FD
COM
Bone infarcts
Pagets
Irradiated bone
In association implants used for fixation
Types:
Central (medullary)
Peripheral (periosteal)
Difference
1- Incidence:
2- Behavior
3- Pathology:
A/ Site:
B/ Macro. :
Cl Micro.:
1) Cells
2) Whorly appearance
3) Bone Trabeculae
4- Clinically:
A) Principle complaint
B) Pain:
C) Path. Fr.
D) Metastasis
5- X-ray:
Central
10% of 1ry bone malignancy
Early destructive
Peripheral
Less frequent
May encircle the whole bone (out NBF)
Metaphyseal
36
Histopathological Grading:
Collagen
Spindle Fibroblasts
Nucleus
Chromatin
Mitosis
Nucleoli
Grade I:
Abundant (Thinner Than Desmoid Tumor)
Grade II:
Thin Collagen
Hyperchromatic Nuclei
Coarse Uneven
Few
May Be Prominent
Differential Diagnosis:
Neoplasm
Non Neoplasm
Metastatic carcinoma
multiple myeloma
MFH
paget
FCD NOF
Reticulo-endotheliosis
Prognosis:
Fibrosarcoma recur rather than metastasize
Like parosteal OS & chondrosarcoma; tend to change their cytological ccc after surgery
10 YSR is 30% in primary FS; and < 10% in secondary FS
Treatment
LG Stage I limb salvaging excision with wide margin
HG StageII radical or wide margin adjuvant chemoradiotherapy
Classically considered radio-resistant
May however be useful as adjuvant to surgery and chemotherapy
Useful for irradiation of lung secondary deposits not accessible to surgery
37
38
39
Clinically
Mostly asymptomatic
Pain & tenderness
Palpable mass at the lesion site
Pathological # (Rarely)
Lesions of the skull
Elicits a more lytic & scalloped appearance
May be associated with a palpable soft tissue mass
Pelvic lesion: subtle hip pain y by movement
X-Rays
Diaphyseal ( NOF metaphyseal)
Oval Mottled lytic defect usually no sclerotic rim
Starts intramedullary and erodes the endosteum as it enlarges
Eventually it erodes the cortex bone expansion & cortical tunneling
Trabeculation: May appear loculated due to sparing of large trabeculae
Periosteal lamellated NBF
Sometimes soft tissue mass is encountered
Flat bones and ribs
lesions appear punched out
Cortical scalloping may occur
Spinal lesions:
Collapse (vertebra plana)
Scoliosis may occur
More in dorsal spine (body or arch) and more in children
Sometimes no localised bone lesion but generalised osteoporosis
99m
Tc
Usually hot (but sometimes cold)
Differential Diagnosis:
1. Ewings: soft tissue mass arising from the tumor lesion
2. Osteomyelitis
3. ABC
4. FD
5. Lymphoma
6. Leukaemia
Treatment
Usually heal spontaneously and need no ttt except when it is large and symptomatic
Intralesional excision and grafting disappointing recurrence more extensive lesions
Wide local excision + BG is recommended
Steroids is another option and tends to result in rapid resolution
Radiotherapy for aggressive or inaccessible lesions (500-1000 rads)
Prognosis
Letterer Siwe disease worst prognosis often fatal in infancy
Hand Schuller Christian : excellent prognosis if there is no extra-osseous disease
Skeletal lesions: only do not death
Soft tissue involvement & rapid course & young age = worse prognosis
Liver involvement 50% die
Lung involvement usually not fatal
Anaemia increased mortality and indicates poor prognosis
40
Cortical Desmoid
(Desmoplastic Fibroma)
RARE tumor of bone the may present as an intramedullary or periosteal tumor. And histologically
Incidence:
< 30Y (70%) .............................................................................................. (unlike fibrosarc)
Pathology:
Gross:
Clinically:
if any, PAIN
Painless SWELLING
Occasionally PATHOLOGICAL #
PXR:
Treatment:
It is more aggressive than other fibrous lesions and Enneking recommended WIDE EXCISION as
the method of choice
If wide excision gonna make a big deal of functional deficit intralesional CURETTAGE + BG
adjuvants:
Phenol
Cryotherapy
NB: GARDNER SYNDROME: Desmoid tumors + osteomas + epidermal cysts + colonic polypi
41
bxzx|v g
o Osteosarcoma
o Osteoid Osteoma
o Osteoblastoma
42
Osteosarcoma
Defiin
niittion:
1ry sarcoma of bone MALIGNANT OSTEOID FORMATION arising from MESENCHYMAL BONE FORMING
cells
It is the commonest 1ry bone sarcoma
Classificatiio
on:
Osteosarcoma
Intracortical
Extra-Skeletal
Conventional
Osteoblastic
Chondroblastic
IM
Telangiectatic
Fibroblastic
Multifocal
Small cell
LG IM
Parosteal
Juxta-cortical
(surface)
Periosteal
Secondary
HG surface
Mixed
I- Intra-Medullary Osteosarcomas
1- Conventional Osteosarcoma
Inciid
dence:
2ND MOST COMMON PRIMARY malignancy of bone behind MM (20% of all 1ry sarcomas)
HG OS progressive pain
LG OS painless swelling
initially may improve with conservative measures and activity modifications
eventually becomes severe if the diagnosis is delayed
Night pain is very important clue (only 25% of patients)
Tender, soft tissue SWELLING
Patients frequently are MISDIAGNOSED with a more common problem at the initial visit
The average DELAY from onset of symptoms to the correct Dx 15 wk :
failure to obtain PXR at the initial visit
failure to repeat the PXR when a patient's symptoms persisted or worsened
43
Pa
Pathological features
Site:
METAPHYSEAL (90%)
Commonest sites: DISTAL FEMUR, PROX TIBIA & PROX HUMERUS
Macroscopic:
GRAYISH FLESHY mass eroding the cortex & the periosteum.
Areas of HEMORRHAGE, NECROSIS & CYSTIC degeneration
May FUNGATE outside the bone & involve the soft tissues
Osteoblastic Osteosarcoma:
1. Malignant osteoid (MO) prevails
2. Arranged as a delicate meshwork or broader confluent masses
3. Calcification of the matrix is variable .
Chondroblastic Osteosarcoma:
1. Malignant cartilage production
2. Still there must be malignant osteoid matrix present to Dx
fibroblastic osteosarcoma
1. Large areas of fibroblasts arranged in intersecting fascicles.
2. Such areas are indistinguishable from fibrosarcomas
3. Thorough sampling is necessary to identify the MO component.
44
Radiological features
METAPHYSEAL lesion (~ 10% are primarily diaphyseal & < 1 % epiphyseal)
MEDULLARY lesion radiodensity & radiolucency
PERMEATIVE invasion poorly defined borders & wide zone of transition
Cortical DESTRUCTION
Periosteal elevation & reaction SUNRAY , HAIR-ON-END, CODMANS
Extra-osseous EXTENSION, and soft-tissue CALCIFICATION
RADIOLOGICAL CLASSIFICATION:
2- Telangiectatic osteosarcoma
RARE - 5% of all osteosarcomas
AGGRESSIVE
Presents with PATHOLOGICAL FRACTURE
May look like ANEURYSMAL BONE CYST
Radiology
MRI
T1 - high intensity with fluid-fluid levels.
Pathology
site: same
Gross
Multi-cystic "BAG OF BLOOD"
May look like ABC
Mic
Large blood filled spaces and thin septa; low power (DDx ABC)
Within the septa there is MO + pleomorphic malignant cells; high power
Prognosis
Poor
45
3- Small-Cell Osteosarcoma
Small cell osteosarcoma, another RARE variant
It is HG LESION that consists of small blue cells that may resemble Ewing or lymphoma.
Cytogenetic and immunohistochemistry studies sometimes are needed to differentiate
Histologically:
In small-cell osteosarcoma the neoplastic cells are round rather than spindle-shaped
Consists of NESTS & SHEETS of blue cells separated by fibrous septa .......... (similar to Ewing)
Cells:
Small round blue cells
Well defined border
Distinct rim of cytoplasm
Round nuclei Fine chromatin
Occasionally, transition to spindle cells is noted
LACE-LIKE OSTEOID MATRIX often surrounds small nests of cells confirms the diagnosis if
present. It may present in only a small quantity, can be difficult to differentiate from the
fibrin-like material that may be present in Ewing sarcoma.
46
1-Parosteal Osteosarcoma
It is a distinct variant of osteosarcoma that arises from the cortical bone characterized by
SLOW growth and LATE METASTASIS
Incidence:
~ 4%
OLDER age group
Has a BETTER OVERALL PROGNOSIS than osteosarcoma (long-term survival rate ~ 75-85%)
Pathology:
Site:
Arise form the CORTEX of DISTAL FEMUR ..............(most common location)
Characteristically, ATTACHED TO POSTERIOR FEMORAL ASPECT
Prox humerus & tibia .......................................(next most frequent sites)
NEA
Tends to form a PROTUBERANT LOBULATED ossified mass
Other areas may INFILTRATE into the adjacent soft tissues
The tumour usually ENCIRCLES (partially or completely) the bone
covered in part by a CARTILAGINOUS CAP (DD. Osteochondroma bone medulla is
not continuous that of the neoplasm)
Microscopically:
It is regarded as a Mesenchymoma i.e. it contains all mesenchymal tissues:
Bone
Cartilage (bluish white)
Fibrous tissue
Even muscles present in peripheral parts (after it invades the surroundings)
Classically LG lesion:
irregular MO trabeculae surrounded by a FIBROBLASTIC spindle cell
There may be foci of ATYPICAL CHONDROID differentiation
HG lesions: y the chance for IM involvement
Clinically:
47
2-Periosteal Osteosarcoma
Periosteal osteosarcoma is a rare INTERMEDIATE-grade malignancy that arises on bone SURFACE
It occurs in a slightly OLDER and broader age group.
Pathology:
Site:
DIAPHYSES of the femur and tibia
NEA
It projects into the adjacent soft tissues as a well-CIRCUMSCRIBED LOBULATED mass
Histologically:
Intermediate-grade chondroblastic OS
The tumour reveals a DOMINANT CHONDROID LOBULES
May contain markedly ATYPICAL CHONDROCYTES.
At the periphery of the lobule the SPINDLE-CELL component produced intercellular MO
Areas of MO and chondroid matrix can be seen to INFILTRATE the cortex
Radiologically
Histologically:
Radiologically
48
49
Incidence:
UNCOMMON ~ 3-4% of osteosarcoma
Pathology
V- Intra-CorticalOsteosarcoma
This is the RAREST osteosarcoma, and the term applies to the lesion arises in the cortex
It was 1st described by Jaffee in 1960 in a report of 2 cases, & through 1991 only 9 cases
Pathology:
Site
INTRACORTICAL in the FEMUR or TIBIA
Measures < 4 cm in diameter
Microscopically:
SCLEROSING variant of osteosarcoma (MINERALIZED MO)
May contain small FOCI of chondrosarcoma or fibrosarcoma.
Radiologically:
Prognosis
50
VI- Extra-SkeletalOsteosarcoma
=Soft-tissue OS is RARE 1.2% of all soft-tissue sarcomas & 4% of all osteosarcomas
>
Clinically:
Sites:
Deep soft tissues of the THIGH ................. (45%)
UPPER extremity ....................................... (20%)
RETROPERITONEAL ...................................... (15%)
Osteosarcomas are usually large ....................... (~10cm)
Histologically:
Variable amounts of MO
Cartilage, fibrosarcoma, MFH, and Schwannoma all are seen (MESENCHYMOMA)
Radiologic
Large soft-tissue masses focal to massive areas of mineralization & z osseous involvement
Pseudo capsule may also be apparent
Scintigraphy
Treatment:
Tumour size is the most important prognostic factor (> 5 cm having a bad outcome)
Despite aggressive therapy, overall prognosis is poor (Death >60%)
Metastases are frequent to Lung, LN, bone
51
Differential Diagnosis
Radiological
Osteosarcoma
Osteolytic
Osteosclerotic
Fibrosarcoma
MFH
ABC
GCT
Brodies abscess
Mixed
Osteoid osteoma
Stress fracture
Osteoblastoma
Ewing
Osteoid osteoma
Stress fracture
Osteoblastoma
OM
Pathological
Osteogenic
1.Osteoid osteoma
2.Osteoblastoma
3.S tress fracture
4. Healing callus
1.NOF
2.Fibrous dysplasia
3.Osteoblastoma
4.Chondomyxoid fibroma
Chondrogenic
Small cell OS
1.Chondrosarcoma
2.Enchondroma
3.Chondroblastoma
1.Ewing sarcoma
2.Plasma cell tumour
ParostealOS
Fibrogenic
1.Myositis ossificans
2.Osteochondroma
1.Fibrosacoma
2.MFH
3. Fibrous dysplasia
4. Chondomyxoid fibroma
Periosteal OS:
Myositis ossificans
Extra-Skeletal
Conventional
Osteoblastic
Chondroblastic
IM
Telangiectatic
Fibroblastic
Mixed
Multifocal
Small cell
LG IM
Parosteal
Juxta-cortical
(surface)
Periosteal
Secondary
HG surface
52
Prognosis of Osteosarcoma
Between 70 - 90% of osteosarcomas in the limbs can be treated with limb-sparing surgery
and chemotherapy so that amputation is not necessary.
When osteosarcoma affects one limb, the LONG-TERM SURVIVAL RATE (LTSR) is 60 - 75%
The prognosis will depend on :
1. Spread
5. Response to chemotherapy
2. Site
6. Type of osteosarcoma
3. Size
7. Serum markers
4. Surgery
8. General health
1- Tumour Spread
Patients localized disease better prognosis ........................5YSR 70%
20% of pts have lung metastases at diagnosis ...........................5YSR 40%
The prognosis for metastatic disease depends on:
Site
53
6-Serum markers
A number of potential prognostic factors were identified and not validated yet these include:
1.Human epidermal growth factor.
2.Tumor cell ploidy.
3.Specific chromosome gains or losses.
4.Loss of heterozygosity of the RB gene.
5.Loss of heterozygosity of the p53 locus.
6.y expression of p-glycoprotein
y AlkPh. at diagnosis are more likely to have lung metastases
y LDH is bad prognostic value
Treatment of osteosarcoma
1. Operative: Which will be tailored according to the stage & extension of the tumour
2. Adjuvant therapy: Preoperative & Postoperative CHEMOTHERAPY
3. Adjuvant surgeries: As resection of a lung metastasis
1. Adjuvant therapy (Chemotherapy)
Grade
Degree of necrosis
Prognosis
Grade I
Grade II
Grade III
Grade IV
<10% Necrosis
<90% Necrosis
>90% Necrosis
100% Necrosis
Bad
Bad
Good
Excellent
Mechanism of action
Side effects
Doxorubicin
(Adriamycin)
54
2. Qperative Treatment:
1- RESECTABLE METASTASIS:
2- INOPERABLE METASTASIS
Chemotherapy
Palliative procedures
LIMB SALVAGE
Wide tumour Resection & Reconstruction of the skeletal gap by Common Modalities Of
Reconstructive Options
Surgical guidelines for limb sparing surgery:
1. BONE should be resected 3-4 cm beyond abnormal uptake as determined by Tc. MRI is useful
2. En bloc resection of normal MUSCLE cuff in all directions
3. Enbloc resection of all previous BIOPSY SITES & potentially contaminated tissues
4. Adjacent JOINT & capsule should be resected
5. There must be enough MUSCLE REMAINING for a functional limb to be reconstructed
6. There must be FREE NEUROVASCULAR bundle of tumour
7. There must be adequate soft tissue COVERAGE
Types:
Limb Salvage
Biological
Reconstruction
Distraction
osteogenesis
BG
Autograft
Allograft
Structural
Osteochondral
Metal
Reconstruction
Intercalary
Reimplantation
Heat
Non
structural
Resection
arthrodesis
APC
Freeze
Composite
Reconstruction
Auto Prosthetic
Composite
Irradiation
Allo Prosthetic
Composite
55
Types:
1. Biological reconstruction
A- Bone grafing
i. Autograft
ii. Allograft
1. Nonstructural allograft
2. Structural allograft
a. Osteochondral allograft
i. Hemicondylar
ii. Total candylar
b. Intercalary allograft.
3. Resection arthrodesis
4. Allograft prosthesis composite (APC)
iii. BG substitutes
B- Distraction osteogenesis:
i. Type 1: diaphyseal
ii. Type 2. Methaphyseal reconstruction
iii. Type 3. Epiphyseal reconstruction
iv. Type 4. Subarticular reconstruction
v. Type 5. Arthrodesis
C- Reimplantation: of the resected bone after devitalization of the tumour cells by:
i. Heat:
1. Autoclaving
2. Pasteurization
3. Microwave induced hyperthermia
ii. Freezing (Liquid nitrogen)
iii. Irradiation
2. Metallurgic reconstruction
3. Composite reconstruction:
A- Auto-prosthetic composite:
56
3. Radiotherapy:
57
Osteoid Osteoma
Introduction
Benign skeletal neoplasm of unknown etiology is Composed Of OSTEOID AND WOVEN bone
The lesion initially appears as a small sclerotic island in a circular lucent defect. (N
NIDUS)
OO may REGRESS spontaneously. The mechanism of involution is not known ( infarction)
Aetiology
1. Congenital.
2. Neoplastic: Jaffe Very slowly growing tumor
3. Rheumatic.
4. Reactive bone lesion (Now) localized reaction to infection
5. Inflammatory (Lymphocytes) sclerosing osteomyelitis :
Classification
1. Cortical
2. Cancellous
3. Subperiosteal
4. Intra-articular
Sites:
Medial Femoral Neck (Juxta- or intra-articular)
Hands and Feet, in particular, the neck of the talus.
58
Incidence
Site:
METAPHYSIS or DIAPHYSIS .........75%
Appendicular skeleton...........65% (FEMUR & Tibia & Humerus; but occur in any bone)
Spine: ....................................12% (Posterior arch)
Hands: ...................................8%
Feet:.......................................4%
The skull and facial bones are involved exceptionally
80% of cases involve the cortical bone; 20% are intramedullary
Gross Pathology
Aggregate of RED, brown, purple or grey tissue (according to vv), GRITTY consistency
Encased in a dense cortical SCLEROSIS
Size of surrounding sclerosis may exceed the size of the principle lesion many times
Nidus does not exceed 2 cm (0.5-2 cm)
Described as a Cherry Piece inside the bone.
Microscopic
Nidus of OSTEOID TISSUE + WOVEN BONE TRABECULAE
HIGHLY VASCULAR CT matrix large dilated vascular channels.
GIANT CELLS and OSTEOCLASTS are frequently observed.
Jaffe described it microscopically to be formed of 3 characteristic regions:
Inner region..............vascular GRANULATION + OSTEOBLASTS.
Intermediate ............. CALCIFICATION & OSTEOID formation
Outer region .............Bone TRABECULAE in various stages of ossification
Clinical picture
Characters:
y activity & at night with congesion & z by salicylates.
Not relieved by rest.
At 1st it is vague & later y in severity & becomes aching in character
Some times, severe enough to waken the patient.
In a few instances pain becomes severe & persistent (6y) threats of suicide
Site:
Usually the pain is at the site of the lesion
Femoral upper end; esp. the neck, may be referred into the leg as a LDP.
Cause:
Result from the lesion being very vascular numerous nerve fibers.
dema & increased tension result in of these nerve endings
59
Osteoid Osteoma Of The Spine
PAINFUL SCOLIOSIS ms spasms abnormal alignment (also kyphosis, torticollis & lordosis)
A may be CONCAVE TOWARD the Lesion.
May be ACUTE and is frequently initiated by PHYSICAL EXERTION.
Osteoid osteoma has been called the most common cause of painful scoliosis.
Osteoid osteoma affecting the hip
MRI
60
Treatment:
COMPLETE EXCISION the WHOLE NIDUS will provide cure
Partial removal of only symptomatic improvement but not cure & will recur
To avoid incomplete removal:
PRE-operative Good x-ray
INTRA-operative x-ray for the removed specimen (more clear than the whole bone)
Remove nidus ENBLOC + Prophylactic ORIF + BG (esp. in neck femur & long bones)
Techniques of osteoid osteoma ablation:
Percutaneously ablated by using radiofrequency (RF)
Ethanol
Laser
Thermo coagulation therapy under CT guidance
If sclerotic bone is removed and not the nidus pain will continue
Metaphyseal lesions its rapid healing; prophylactic ORIF is usually not necessary
Spinal lesions sometimes complete ablation & resection are not feasible
Ivory Osteoma
= Compact osteoma = Ivory exostosis
Rare benign tumor arise as a localized thickening on the outer or inner surface of bone
Sites: usually in skull and medial tibia
Clinically: painless, hard lump, on the outer surface of the skull
If occur on the inner surface epilepsy
PXR: sessile dense bone well circumscribed edge
TTT: only when symptomatic
61
Osteoblastoma
Introduction
Osteoblastoma (OB) is an UNCOMMON primary neoplasm of the bone. It has clinical and
histologic manifestations similar to osteoid osteoma (producing osteoid, primitive woven
bone & fibro-vascular CT); therefore, some consider the two lesions to be variants of the
same disease, with osteoblastoma representing a giant osteoid osteoma. However, an
aggressive type of osteoblastoma has been recognized, making the relationship less clear
tiology
UNKNOWN.
Incidence
Site
40% in SPINE (17% of spine OB are in the SACRUM)
Posterior element
DIAPHYSES is another common site (LL more affected)
Sometimes Metaphyses are affected
Rarely epiphyses are affected
Pelvis, hands, feet, skull, clavicle, scapula, ribs, and
talus are also documented
Macroscopically
IRREGULAR OSTEOID mass
MORE VASCULAR, MORE GRITTY, but LESS SCLEROTIC than osteoid osteoma
Microscopic
Most important: IMMATURE BONY TRABECULAE & primitive WOVEN bone ossification
Lined OSTEOBLASTS
HIGHLY VASCULARIZED CT: The stroma has dilated capillaries & blood sinusoids
NIL MITOTIC ACTIVITY
62
X-RAY
Spine Osteoblastomas:
In the POSTERIOR ELEMENTS
WELL-DEFINED expansile lesion
50% contain MATRIX MINERALIZATION.
Long bones lesions:
66% in the cortex & 33% in medullary canal.
> 2 cm unlike osteoid osteoma
Well-circumscribed radiolucent lesion
Thin shell of peripheral NBF & reactive zone of bone
Computed tomography (CT):
Provides information about the extent (NB. MRI findings alone are not diagnostic)
Detects soft tissue changes
Detects BM extension
Bone scintigraphy
Differential diagnosis
ABC
Chondromyxoid Fibroma
Enchondroma and Enchondromatosis
EG
Pain
Size
Grossly
Mic
GCT
Osteoid Osteoma
Osteomyelitis
Osteosarcoma
Osteoid Osteoma
Osteoblastoma
More
< 2 cm whatever the duration
Only sclerosis of neighboring bone
Less osteoblasts
Less
Increase with long standing up to 7 cm
Some destruction & expansion of neighbor bone
More osteoblasts
Treatment
63
V{wzx|v g
OSTEOCHONDROMA
o MULTIPLE HEREDITARY EXOSTOSIS
o ENCHONDROMA
o CHONDROBLASTOMA
o CHONDROMYXOID FIBROMA
o CHONDROSARCOMA
o
65
Osteochondroma
Osteochondroma is the MOST COMMON BENIGN BONE TUMOR. Mostly
asymptomatic, but they can cause mechanical symptoms
CARTILAGE CAP histology has the same defined zones in physis, namely
zone of proliferation, hypertrophy, calcification, and ossification
Incidence:
35% of benign bone tumors
10% of all bone tumors
< 20 years
:=3:1.
Etiology:
UNKNOWN; although, it was thought to be a PHYSEAL HERNIATION
Site
Any bone that undergoes endochondral bone formation.
FEMUR .....................................30% / distal: proximal=3:1
Tibial ....................................20%
Proximal Humerus ...............20%
Hands and feet (10% of cases), scapula (4%), pelvis (5%)
Location:
Osteochondromas develop METAPHYSEAL ADJACENT TO PHYSIS
Then, grow AWAY FROM PHYSIS (as if isolated physis):
Rate of epiphyseal cartilage growth > osteochondroma
The muscle pull (even if not attached to it)
Growth of the osteochondroma STOP AT SKELETAL MATURITY
Macroscopic
May have a stalk PEDUNCULATED, or a broad base SESSILE
The stalk is made up of mature bone.
By definition, medullary canals of the bone and the stalk are
CONNECTED
CARTILAGE CAP - is thick in children- is replaced by endochondral bone at maturity
Microscopic:
Lesion is topped with a cartilage cap VARIABLE CELLULARITY.
The cap has an overlying fibrous layer MESENCHYMAL CELLS responsible for its growth
Cap cells are VERTICALLY oriented as in physis
Staging: Musculo-Skeletal Tumor Society (MSTS) staging for all benign lesions:
Stage I .................................. Inactive or static lesions (Latent)
Stage II ................................. Actively growing lesions (Active)
Stage III ............................... Actively growing & locally destructive (aggressive)
66
Clinical picture:
most commonly diagnosed INCIDENTALLY when PXR obtained for some other reason.
The second most common presentation is a MASS ( pain)
CAUSES OF PAIN IN OSTEOCHONDROMA:
1. Pressure on the overlying soft tissue.
2. Bursitis over the exostosis.
3. Irritation of surrounding tendons, muscles, or nerves can result in pain.
4. Stalk fracture
5. Cap infarction
6. Malignant transformation.
Complications:
1.
2.
3.
4.
Fracture
Neurologic Sequelae
Bursa Formation
Malignant Transformation
Fracture
Site: typically involves the stalk base esp. around the knee
Subsequently, callus formation causing band like sclerosis on PXR occurs healing.
No significant incidence of nonunion has been reported.
Interestingly, REGRESSION of solitary osteochondroma occur spontaneously and following #
Neurologic Sequelae
Peripheral lesions may compress nerves .... nerve palsy
PERONEAL affection foot-drop & RADIAL nerve Palsies are reported
Also central neurological manifestations are reported also central lesions
Bursa Formation
The commonest locations: (sites motion & friction):
SCAPULA ............................................ 50% of shoulder cases
LESSER TROCHANTER
1n certain bones, such as the pelvis and scapula useful to localize the lesion
67
MRI
of malignant degeneration
Treatment:
68
Diaphyseal Aclasis
AD
65-75% have MUTATIONS at EXT1 gene & ~ 21-30% at EXT2 gene
Peak at 3y (nearly all patients are diagnosed by 12y)
HME Findings y with:
AGE: at birth5% & 12 Y96% & adult75% have an evident deformity
MALES > females.
HME Malignant risk y age
Pathology
DEFORMITY:
ABNORMAL REMODELING shortening, bowing, widening of metaphyses
Radius &Tibia
BOWING of the forearm or leg, is the commonest y Ulma
& Fibula deformities
69
PXR
Clinically:
Pain, swelling
Rarely claudication
Palpable pulsatile mass usually affecting a young patient.
70
71
Enchondroma
Benign tumour of cartilage originating within the medullary cavity
Periosteal form originates in the periosteum and erodes into the cortex
Incidence
Pathology
Site:
o
o
o
o
Clinically
Radiology
Treatment
Prognosis
72
Chondroblastoma
Incidence
Pathology
Clinically
X-Rays
OPEN PHYSIS
ECCENTRIC, EPIPHYSEAL, WELL DEFINED, LYTIC area placed in the or across the physis
Differential Diagnosis:
GCT (adults)
ABC (histology similar)
clear cell chondrosarcoma
epiphyseal osteomyelitis
Treatment
Prognosis
73
Chondromyxoid Fibroma
Incidence
Pathology
MNGC
MACROPHAGES
MONOCYTES with condensations of cells on the periphery
Usually NO BONE OSTEOID
Clinically
X-Rays
Treatment
Prognosis
74
Chondrosarcoma
Incidence
th
Pathology
Sites:
1. PELVIS ....................................30%
2. Prox Femur ..........................20%
3. Prox Humerus ......................10%
4. Ribs ......................................10%
5. Rare in hand but it is the commonest sarcoma there
6. Often metaphyseal
Conventional Macroscopic appearance:
o EXPANDING lesion cortical DESTRUCTION, soft tissue extension
o Grayish HYALINE FUSED NODULES areas of CALCIFICATION
Conventional Microscopic appearance:
o Cellular PLEOMORPHISM + focally CALCIFIED matrix
Histological Classification:
Grade I
(30-35%)
Grade II
(40-50%)
Clinical Behavior
SLOW GROWING
LOCALLY AGGRESSIVE
Metastasis in 20%
Metastasis in 70%
Chondrocytes
Matrix
Low cellularity
Abundant matrix
Calcification
More cellular
Less Matrix
Focal myxoid change
Intense hypercellularity
Sparse matrix
Topographic Classification:
1. INTRAMEDULLARY either DENOVO or 2RY to enchondroma:
Olier's & Maffucci's are at much higher risk
2. SURFACE usually 2RY to osteochondroma:
y SIZE, FUZZY, CAP > 4cm, BASE > 6cm diameter
Pathological Classification:
1. CONVENTIONAL
2. LOW GRADE:
3. HIGH GRADE:
1. MESENCHYMAL CS
2. DEDIFFERENTIATED CS
Metastasize to lung
75
Clinically
X-Rays
Variable appearance with 60 - 70% have calcification and 50% have sub periosteal NBF
Large CYSTIC lesion cortical DESTRUCTION + SCALLOPING & EXPANSION
CENTRAL CALCIFICATION
Treatment
These tumours tend to METASTASIZE LATE therefore attempt wide local excision initially
Radiotherapy useful for the treatment of surgically inaccessible sites however are relatively
CHEMO & RADIO-RESISTANT
Prognosis
Dependant on grade:
Low grade ........................................80% 5YSR
High grade........................................20% 5YSR
DEDIFFERENTIATED CHONDROSARCOMA
HG chondrosarcoma + areas of osteosarcoma, fibrosarcoma, or MFH (in this frequency)
Rare HG
Usually occurs in the RIBS or JAW
Age usually 10 - 30 years
Sheets of small undifferentiated cells resemble HG EWINGS + focal LG CHONDROID matrix
Metastasis usually to lung
77
V|v _x|
(1) Simple Bone Cyst
(2) Aneurysmal Bone Cyst
(3) Giant Cell Tumor
79
to an area of bone growth in the physeal line creating a defect at that point failure of
endochondral bone formation resulting in a hiatus present as a unicameral bone cyst.
In favor of this theory ........................... age group affected.
Against this theory ................................ cysts that cross the mid-diaphyseal point
H EMORRHAGIC THEORY
Some suggest that solitary cyst is the result of a hmorrhage into the metaphysis
organizes by fibrous wall formation; while the blood volume clots then liquefies
The surrounding fibrous capsule acts as a semi-permeable membrane & if the osmotic
pressure of the intra-cystic content is higher than that of the surrounding tissue, water is
drawn into the cyst y intra-cystic pressure and the cyst expands pressure erosion on
the surrounding cancellous tissue & then cortex.
Extra & intra cystic densities ................ eventually stabilizes latent stage
Against this theory ................................ pts hemophilia do not show a y incidence of SBC
FAILURE OF BONE FORMATION :
If the cyst is a defect resulting from failure of bone formation by an aberrant part of the
physis defect involves the diaphyseal portion supposed to be formed by that part
The cartilage growth is no longer converted to bone; while the surrounding bone is
remodeling normally false appearance of expansion that never been wider than the
epiphyseal line & this is a diagnostic feature.
NBF laid down as the child matures & the cyst then occupies an area towards diaphysis
In adult state many cysts are obliterated radiographically by NBF on the overlying cortex.
It becomes smaller than the width of the adult bone
80
Pathology
SITE
Tubular bones ...................................... 90-95% of patients.
Location ................................................ PROXIMAL METAPHYSEAL
Diaphyseal in only ............................... 4-12% of patients
Commonest site..................................... PROXIMAL HUMERUS & UPPER FEMUR.
MACROSCOPIC:
There is always ..................................... ZONE OF NORMAL BONE between the cyst & physis
represents the amount of longitudinal bone growth since the establishment of the lesion.
It has been stated that cysts that appear very near the line are still actively growing & that
therapy at this time ............................... result in a RECURRENCE
As the bone continues to grow.............. CYST DRIFT to mid-diaphysis (never cross it)
The outer cortical shell ......................... covered by an intact periosteum
The cyst contains ................................. CLEAR SEROUS FLUID (Occasionally, blood if #)
The cyst is lined by .............................. PALE BROWN MEMBRANE of few mm thickness
After a fracture ..................................... Fibrous septa form MULTILOCULAR APPEARANCE.
MICROSCOPIC:
There are no pathognomonic features about the tissue that is curetted from the bony walls
Lining membrane is formed of ............ young fibrocytes + deeply stained fibrin + collagen
Hemosiderin pigments
Xanthoid cells
MnGC
Clinical picture:
X rays
81
Differential Diagnosis:
Fibrous dysplasia.
Non-ossifying fibroma.
Giant cell tumour.
Enchondroma.
Osteitis fibrosa cystica.
Neurofibroma.
Treatment:
Surgery is indicated primarily:
o
o
o
Modalities:
1. Traditionally.......................................... CURETTAGE + BG
Recurrence that require redo ........... 25%.
Recurrence z with ......................... high-speed burr on the cyst walls after curettage
Cauterization with phenol following curettage has not reduced the recurrence.
2. Currently successful healing is reported by METHYL-PREDNISOLONE ACETATE (200 mg)
Common............................... 50%
Callus of repair .................... exerts healing influence
Considered as ...................... diagnostic & curative
Malignant transformation ............... very rare
o
o
o
82
Etiology:
Vascular malformations
AV fistulas
Venous blockage
Altered hemodynamics
The vascular lesions y pressure / expansion / erosion / resorption of bone
Local hemorrhage formation of reactive osteolytic tissue
o
o
o
o
Pathology
SITE:
ABCs may affect any bone in the body
ABCs most common ............................ 80%LONG BONES spine flat bones (pelvis)
Mainly................................................... ECCENTRICALLY METAPHYSEAL
subperiosteal, diaphyseal, epiphyseal ... rare
2ry lesions tend to occur in the sites where 1ry lesions typically arises.
83
MACROSCOPIC APPEARANCE:
Cystic blood-filled cavity charcteristic BLOOD-SOAKED SPONGE appearance
marked expansion or ballooning of the bone
The cavity is divided into cavernous spaces by intertwining fibrous mesenchymal septa
Thin subperiosteal shell of NBF surrounds the lesion
Intra-op.: when blood is sucked refill quickly (WELLING UP) = (POURING OF BLOOD)
It has no connection systemic circulation; but its blood comes from septal capillaries
MICROSCOPIC APPEARANCE:
Large vascular spaces
Fibrous septa contain:
Plain X ray:
Q:
Central metaphyseal
Entire segment of a bone
Eccentric metaphyseal
Subperiosteal diaphyseal
Metadiaphyseal
Cortical Expansion
Cortical Erosion
Min
Marked
Min
Min
Marked & push periosteum to soft tissue
+/+
+
CT scanning:
Same PXR finding
Internal SEPTATION (ie, calcified rim, eggshell appearance)
FLUID-FLUID LEVELS; these are most often found in the ABC, but they are not exclusive to it.
84
MRI:
As CT scan, but they can more specifically reveal blood & expansion into soft tissues
The appearance on imaging studies has been divided into 4 PHASES OF PROGRESSION:
Phase
Progression
Cortical Expansion Cortical Erosion
Trabeculation
I- Initial (incipient)
II- Growth
-Starts slow
-Rapid
III- Stable
-Plateau
IV- Healing
-Progressive calcifn
-Little
-Massive destruction
& bone lysis
-Classic
-Min
-Destruction outpace
trabeculation & NBF
-Numerous
-Healing
-Coarse irregular
ABC
Young
Metaphyseal Eccentric
Cavernous blood spaces
fibrous septa + Giant cells
Blood-soaked sponge
Ballooning
Adult
Epiphyseal central may extend to metaph.
Spindle cells
Giant cells + Bl. vessels
Liver piece
Lack of expansion
ABC
SBC
young
Eccentric Metaphyseal
Blood-soaked sponge
Ballooning
Egg shell
younger
Central Metaphyseal then diaphyseal growth
Clear yellow fluid
Cortical endosteal erosion & expansion
Cortical continuity
SBC
Age
Site
Macroscopic
Telangiectatic osteosarcoma
the lesion through a cortical window by carefully abrading all surfaces with a high-speed
burr and possibly local adjuvants such as phenol, PMMA, or liquid nitrogen
The adjuvant therapy extends the area of treatment beyond that which physically can be
excised. The use of liquid nitrogen, phenol, and polymethylmethacrylate may achieve an
extended area of treatment. The adjuvants involve the use of chemical, freezing, or thermal
means to cause bone necrosis and microvascular damage to the walls of the physically
excised cyst, disrupting the possible etiology.
85
excision (e.g. extensive bony destruction) and has a recurrence rate of about 7%.
RECONSTRUCTIVE options after wide excision include:
Structural allografting
Autografting
Reconstruction with either endoprosthesis or APC.
IV. ABC in huge lesions of spine and pelvis:
This is an exception for the rule of irradiation to malignant tumors only
Irradiation is reported to induce healing using megavoltage of 30Gr
Operative intervention could be very difficult & associated massive fatal bleeding
Liquid nitrogen
Is the most popular adjuvant. After the ABC is exposed and a window is opened, liquid
nitrogen is poured in the cyst through a funnel
The surgeon should be sure to leave the window open, allowing the gas to escape.
A total of 2-3 cycles of freezing & thawing should be used to obtain maximum necrosis
Surrounding tissue, especially the bundle should be protected
Phenol:
Less used because of its poor penetration of bone compared with that of liquid nitrogen
However, phenol has had some success & is easy to use
Phenol is simply applied by soaked swabs, and remaining phenol is sucked
Cavity is filled absolute alcohol isotonic sodium chloride solution.
Polymethylmethacrylate (PMMA):
Induce bone necrosis from its thermal properties has been questioned in the literature
However, PMMA has the benefit of rendering a large lesion mechanically sound
If used subchondral joint surface should be protected by cancellous grafts or Gelfoam
Indications of PMMA:
1. Huge cavity
2. Old age
3. Weight bearing bone
Selective arterial embolization:
By using angiography, an embolic agent is placed at a feeding artery to the ABC, cutting
off the nutrient supply and altering the hemodynamics of the ABC.
Various materials, such as springs and foam, have been used to create the emboli:
Selective arterial embolization is indicated in:
1. Difficult locations
2. May be performed within 48 hours prior to surgery to z hemorrhage
PROGNOSIS:
The prognosis for an ABC ................... generally EXCELLENT.
The most common problem ................. RECURRENCE
The overall cure rate ............................ 90-95%.
86
Recurrence
st
detect early recurrence & to make sure that the tumor does
not cause deformity or interfere with growth.
Recurrence y :
Capanna I , II ............................. 24%
Capanna III, IV, V ..................... <5%
younger age & open growth plate
Metaphyseal lesion
Stage 3 aggressive lesions
ABC
-15y female
-Unknown cause
-Eccentric Metaphyseal long bone
-Blood soaked sponge
-Welling of Blood
-CP: Pain, Pathological #, Mass
-PXR: Soap Bubble, Egg-shell,
Pencil in cup, Ballooning
-Capanna PXR classification
-MRI Fluid-fluid level, classification
-ttt: Excision (accordingly) + BG
Occur typically in middle-aged men at the ends of long bones, particularly the distal tibia.
Considered intra-osseous extensions of soft tissues ganglia
Subperiosteal ganglia have also been reported .
Treatment is by local excision of overlying soft tissues & curettage of the involved bone .
Recurrences is unusual
87
1.
2.
3.
4.
Traumatic theory
Neoplastic theory
Chronic inflammation
Localized form of parathyroid osteodystrophy
Incidence
5% of all tumors
20 % of all benign bone tumors
Race: Affects all races esp. .................. China & southern India
Sex:
> (Unlike the majority of osseous neoplasms)
In contrast, malignant GCT is more in men (3:1)
Age:
The vast majority ...................... SKELETALLY MATURES
80% ........................................... 3RD DECADE (20-50y)
< 14y ......................................... rare (1-3%)
> 50y .......................................... 10%
Pathologic Features
LOCATION
ECCENTRIC META-EPIPHYSEAL
85-99% of lesions reach .................1 CM SUB ARTICULAR (most important diagnostic feature)
Site of origin is controversial .........METAPHYSEAL SIDE OF THE EPIPHYSEAL PLATE.
MACROSCOPIC
88
MICROSCOPIC
Contains diffuse OSTEOCLASTIC GIANT CELLS, hence the old name osteoclastoma. Giant
cells may suggest the Dx, but not specific; as many lesions have similar appearance.
Dx need clinical & PXR & histologic evaluation of mononuclear component
GCT = large of GIANT CELLS IN A BACKGROUND OF MONONUCLEAR CELLS
MULTINUCLEATED GIANT CELLS (MnGC):
There are ...................... 30 Giant cell/HPF
Cell contains ................. 30 nuclei
Cell diameter ................ 30
Distributed throughout the lesion & their concentration
varies from tumor to tumor. Some tumors have many,
whereas others have a few MnGC nestled in swirls of
spindle-shaped stromal cells
MnGC Concentration.... not related to recurrence or metastasis
MnGC invade vessels .... 3% (although aggressive, not related to prognosis)
MONONUCLEAR cells:
Round, oval, or polygonal & may RESEMBLE HISTIOCYTES
Fusion of these mononuclears may result in giant cell formation
Mitotic figures may be abundant
STROMA:
VASCULAR numerous thin-walled capillaries, often small areas of hge.
Stromal cells NUCLEI ARE INDISTINGUISHABLE from MnGC (helpful in DDx)
May be associated 2RY ABC but solid areas of classic GCT
Small FOCI OF OSTEOID matrix (never chondroid) produced by the stromal cells
Extensive hemorrhage, pathologic fracture, or previous surgery can alter
significantly the usual histologic picture of GCT resemble a 1ry sarcoma
According To the Predominant Growth Character 5 Types Are Present:
1.
2.
3.
4.
5.
SPREAD
Nonspecific and include (in order of decreasing frequency) pain, swelling, z ROM
PAIN is usually of several months duration / z by rest.
Related to associated PATHOLOGIC # acute onset of pain (10%)
NEUROLOGIC symptoms may be associated with spine lesions
89
Differential Diagnosis
DDx of GCT is extensive, including (but not limited to):
Brown tumor
Osteoblastoma
Chondroblastoma
ABC
NOF
Osteosarcoma giant cells.
These lesions can be difficult to distinguish from one another, particularly FNAC or with frozen
section specimens careful clinical, pathologic, and PXR correlation. This is particularly true of
brown tumor of hyper PTH, which can be indistinguishable from GCT. Laboratory analysis (Ca, ph,
& PTH levels) should be performed to exclude this possibility
Staging,
Grade
Behavior
MnGC
GI
G II
G III
Benign PXR
Aggressive PXR
Metastasis
15
70
15
z & small
z
Numerous
y
Absent
Moderate + Atypia
Marked atypia + pleomorphism
Grade
Behavior
Margin
Cortex
GI
G II
G III
Latent
Active
Aggressive
WELL DEFINED
RELATIVELY WELL DEFINED, NOT SCLEROTIC
ILL DEFINED
Intact
Thin expanded
Eroded
No correlation exists bet. grading systems and the incidence of recurrence or metastases
DNA analysis has shown only limited value in prediction the behavior of GCT
Recent studies with CT & MRI improved evaluation of soft tissue extension more
reliable data for staging GCT and predicting their clinical behavior
PXR
Bone Scan
y Tc
static uptake in the vast majority of GCT
y uptake peripherally photopenia centrally DONUT SIGN (60%)
99m
Blood pool imaging, dynamic Tc
scintigraphy & Ga67 imaging reveal y uptake; but less
99m
90
Angiography
Angiography of GCT is only infrequently performed since the advent of CT and MRI:
NB
Treatment
1- BENIGN GCT
Depends on:
1. Location of the tumour.
2. Size of the tumour.
3. Tumour grading.
4. Soft tissue extension & articular involvement Joint preservation is impossible
Benign GCT
Excision
Alone
Curettage
Reconstruction
Prosthetic
Bone Graft
Bone Cement
Biological
Distraction
BG
Curettage
Intralesional EXTENDED CURETTE is a limb-sparing option that offers good functional outcomes
Simple curettage BG ......................... RECURRENCE rates of 30-50%
The entire tumour cavity....................... must be adequately exposed for thorough curettage
A large CORTICAL WINDOW ..................... necessary to expose the entire tumour
The intraosseous tumour bulk is removed with a large curette till normal-appearing bone
The cavity is then enlarged ................... HIGH-SPEED BURR (5mm) in all directions + ADJUVANT
High speed burr
Not only MECHANICALLY REMOVE remnants; but also adds a THERMAL NECROSIS for the tumour
Burring of the cavity then
FollowedBy
91
Disadvantages:
92
93
4- MALIGNANT GCT
Malignant giant cell tumour is a term used to describe a heterogeneous group of giant cellcontaining lesions that are capable of MALIGNANT BEHAVIOR and PULMONARY METASTASES.
Prevalence: .......................................... 5%-10% of all GCT
Site: ....................................................... distal tibia and sacrum.
Types:
Primary malignant GCT (de novo lesions)
Extremely rare
Occur most frequently in recurrent cases.
Secondary malignant GCT: (Radiation sarcoma)
Are sarcomas that occur at the sites of
previously treated GCT by DXT
Treatment:
Surgery alone
Surgery and chemotherapy.
Radiotherapy alone
`|vxtx
o ADAMANTINOMA
o CHORDOMA
o HEMANGIOMA
97
Adamantinoma
An EXTREMELY RARE, locally aggressive, osteolytic, LG malignant tumour of epithelial origin of long
bones. It is not related to adamantinoma or ameloblastoma of the mandible and maxilla which is
derived from Rathke's pouch
Incidence
Pathology
Osteofibrous dysplasia (OSSIFYING FIBROMA) has a striking predilection for the tibia and has
well documented association with adamantinoma and may be a benign precursor to it
Site: tibia .............................(90%)
Gross:
o GRAYISH white RUBBERY mass areas of HGE and necrosis
o Bone SPICULES and CYSTS filled blood or straw-colored fluid
Microscopic
o Biphasic tumor...........epithelioid cell islands + surrounding reactive fibrous stroma
o The stroma consists of spindle cells producing collagen
Presentation
Radiology
The lesion may break through the cortex and extend into soft tissue
MRI helps demonstrate the intraosseous and extraosseous involvement.
Osteofibrous dysplasia
fibrous dysplasia
ABC
chondromyxoid fibroma
chondrosarcoma.
Treatment
Prognosis
May METASTASIZE to Lungs, LN, Liver by both haematogenous and lymphatic routes
Survival metastasis............. 12 Y
Local recurrence ................... 20%
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Chordoma
Incidence:
Age .....................................................40-70Y
M:F .....................................................2:1
Aetiology:
nucleus pulposus
The prevailing theory is that in chordomas the notochord fails to
degenerate and then undergoes malignant transformation.
Against this; normal notochord remnants have never been observed!
Pathology
Sites:
o
o
o
o
Gross
o
o
o
SACROCOCCYGEAL region............50%
SKULL BASE.................................30%
Clincal
Sacrococcygeal tumours:
o Low back PAIN (no characteristic pattern or course)
o Bowel and bladder DYSFUNCTION
o Often large at presentation & can be palpated on PR
Other sites:
o DYSPHAGIA .................................Anterior cervical tumours
o NEUROLOGICAL DEFICITS ..............Posterior cervical tumors
o HEADACHE .................................Base of the skull
Radiology:
PXR: solitary mid-line LYTIC lesion + bony DESTRUCTION + soft tissue MASS + focal CALCIFICATION
CT & MRI: demonstrate soft tissue + epidural extension + calcification + identify recurrence
Chordomas have reduced uptake on bone scan.
Treatment
WIDE EXCISION ........................................... (rarely feasible the anatomic location of the tumor)
Sacrococcygeal tumor............................ sexual & sphincter dysfunction after surgery
RADIATION ................................................. if complete resection is impossible
CHEMOTHERAPY ........................................ for late stage disease.
Prognosis:
99
Haemangioma Of Bone
Features
Site
Haemangiomas are hamartomas characterised by vascular spaces lined with endothelial cells
Common, ~ 10% of autopsy cases having vertebral haemangiomas
M:F .....................................................1:2
Age......................................................30 - 50 Y
VERTEBRAL BODIES (thoracic) ................50%
Calvarium............................................20%
Also tibia, femur and humerus
Pathology
GROSS: Vascular HAMARTOMA with CYSTIC, DARK RED CAVITIES (DDx: ABC, telangiectatic OS)
4 types:
o Capillary
Most Common
o Cavernous
o Arteriovenous: remnants of foetal capillary beds
o Venous
MICROSCOPY : Non-vascular components: fat, smooth ms, fibrous, bone, haemosiderin & thrombus
Presentation
Radiology
PXR
Body lesions .......................................POLKA DOT as the vessels are seen in cross section
Calvarial lesions .................................lytic radiating WHEEL SPOKES
Metaphyseal or epiphyseal .................lytic lesions spiculated pattern of "IRISH LACE"
CT
MRI
Treatment
Asymptomatic.....................................No ttt
calvarium Lesions ...............................resected thin margin of normal bone
Vertebral lesions .................................radiation or excision preceded by embolisation
Metaphyseal lesions ...........................excision + BG
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fy g|x ftvt
o PERIPHERAL NERVE TUMOURS
o SYNOVIAL TUMOURS
o FIBROUS TUMOURS
o MUSCLE TUMOURS
o VASCULAR TUMOURS
o FATTY TUMOURS
o RARE SARCOMAS
o BONE METASTASIS
103
Staging
Synovial Tumours
Fatty Tumours
Diagnostic Clues
Fibrous Tumours
Rare Sarcomas
INCIDENCE
Benign soft tissue tumours common
Malignant soft tissue tumours rare
Pathological Classification:
1. Benign
2. Malignant
3. Reactive tumour like lesions
Tissue of origin
Benign
Malignant
Diagnostic Steps
Muscle Tumours
Reactive lesions
104
DIAGNOSTIC CLUES
Size
A small mass < 5 CM in its greatest dr ............unlikely to be malignant
Mass that is > 5 cm ........................................20% chance of being a soft tissue sarcoma
The size of the lesion can be determined by physical examination if the lesion is
subcutaneous and easily palpable, or by ultrasound, computed tomography (CT) or magnetic
resonance imaging (MRI).
Site: Superficial or deep?
Superficial lesions are more likely.................. benign or malignant better prognosis than deep
The depth is best determined by ....................physical exam, ultrasound or MRI.
The THIGH and BUTTOCKS are the two most common sites for soft tissue sarcomas. Any large
deep mass in the thigh or buttocks should be considered at high risk for being a sarcoma
Consistency
Soft tissue sarcomas tend to be ...................... FIRM and NOT VERY PAINFUL until they get very
large and compromise their vascular supply or adjacent neural structures.
Lipomas are usually ....................................... SOFT and NON TENDER
Infectious and inflammatory .......................... WARM and TENDER
Pseudoaneurysm............................................. PULSATILE AUDIBLE BRUIT on auscultation.
Cystic or solid
Most cystic lesions are .................................... INFLAMMATORY or BENIGN, as ganglion & abscess
Solid lesions is either ...................................... benign or malignant neoplasm.
Attempt Transillumination
If deep ultrasound or MR scan will determine this
Duration
A mass that has rapidly increased in size over two months is more likely to be a sarcoma
than the lesion that has slowly enlarged over a 20-year period.
A mass that increases and decreases in size is usually a cystic lesion.
However, caution should be taken with masses that have been present for a long time. Soft
tissue sarcomas occasionally present with a history of many years duration up to 30 years.
PXR
Every soft tissue mass that is going to undergo intervention should have a plain radiograph
Fat density lesion............................................. lipoma
Punctate calcifications..................................... synovioma, chondrosarcoma, hemangioma
Ossification...................................................... osteosarcoma or myositis ossificans
skeletal abnormalities...................................... osteomyelitis, primary bone lesion or periosteal
reaction from the soft tissue tumor
MRI
The MRI gives the most information of any radiographic study but should be reserved for
large lesions or those that are ill defined.
It will clearly delineate whether the lesion is a bone lesion with a very large soft tissue
component (bone malignancy) or whether the lesion is a primary soft tissue lesion.
z Tl & T2 .....................................................either an extra-abdominal desmoid tumor,
extensive scar tissue, cortical or dense bone or a foreign material such as bone cement or air.
y T1 & T2 .....................................................likely a lipoma.
z T1 & T2 ....................................................low-grade liposarcoma & any neoplastic lesion;
benign or malignant.
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DIAGNOSTIC STEPS
History
Examination +/- transillumination
Ultrasound and plain radiography
If lesion< 5cm, cystic and subcutaneous ........... observe
(If patient keen on removal use a longitudinal incision with good haemostasis, ensure the
incision can be encorporated in any later excision and perform an excisional biopsy)
If lesion >5cm, not cystic, or painful.................. MRI, then incisional biopsy / trucut needle
biopsy / fine needle aspiration (see principles of biopsy)
99m
If a possibility of malignancy ............................. Tc
, CXR, Chest CT, Abd CT, CBC, ESR
TREATMENT
Soft tissue sarcoma treatment ............................ resection, amputation, DXRT or a combined
Systemic control includes ................................... chemotherapy is controversial because it has
not been very effective in improving survival & has significant patient morbidity & cost.
PROGNOSTIC FACTORS
1. Stage of the patient (Ennekings stage)
o Metastasis is most frequently identified in the lungs LN skeleton.
o The standard staging studies include a physical examination of LN, CXR, CCT, Tc,
and gallium scan.
o A patient non-metastatic disease at presentation has a far better prognosis
2. Histologic grade.
o Patients HG lesions have a worse prognosis than patients LG lesions.
3. Size of the lesion
o Although small lesions (< 5 cm) are rarely malignant, when they are, they have a
better prognosis than larger lesions.
4. Depth of the lesion
o Superficial (subcutaneous) soft tissue sarcomas have a better prognosis than deep
(below the muscle fascia) lesions.
TIOLOGY:
1. Genetic: e.g. mutations, translocations, deletions, monosomy, trisomy as in NF1
2. Radiation: induce mutations
3. Carcinogenic: e.g. thorium, vinyl chloride
4. Lymphedema: predispose to lymphangiosarcoma
5. Infection: HPV 8, Epstein-Barr virus
6. Trauma
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107
11. Pathological Findings
HISTOLOGICALLY:
1.
2.
3.
4.
5.
6.
Perineural fibrosis
Thickened vasanervosa hyalinized walls
Neuronal edema
Neuronal demyelinization and degeneration
No inflammatory changes
Frequent presence of bursal tissue
ELECTRON MICROSCOPY:
Neuralgic PAIN:
rd
nd
th
o Plantar pain at 3 interdigital space (some times 2 but 4 is rare)
o y walking
o y at night sometimes
o z on removing shoes
TINGLING, NUMB, BURNING, or "DEAD" toe
Vague FOREFOOT tingling
COLOUR changes
The condition may remain undiagnosed for many years.
Clinical assessment
The diagnosis is often strongly suspected within the first minute of the consultation.
Ask about:
PN ....................................................DM and chronic inflammatory disorders
Foot Trauma
Ankle Discomfort ............................may suggest tarsal tunnel syndrome
Spinal problems ...............................history of root entrapment symptoms.
Examination
Any nerve entrapment in spine, proximal limb or tarsal tunnel.
On local examination look for:
o Local TENDERNESS
o SWELLING in the intermetatarsal space
o MULDER'S CLICK or crunching sensation + pain
o LOCAL ANAESTHETIC injection into the affected space
o whole foot should be examined for any cause of METATARSALGIA
A, Squeezing forefoot just proximal to metatarsal heads between index finger and thumb
B, Simultaneous compression of the suspected web space two fingers of opposite hand
Imaging
US & MRI have been described for imaging a neuroma, but we have not had any success
If there is a suggestion of other forefoot pathology PXR should be obtained.
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Treatment:
1.
2.
3.
4.
5.
OPERATIVE TREATMENT:
The dorsal approach is the most common and widely used surgical treatment for interdigital
neuroma. Overall, one can expect 90% good and excellent results with this approach
Under tourniquet, make 3 cm incision starting in the involved web space (usually 3rd)
Place the incision between the metatarsals to avoid injury to a small dorsal sensory nerve
Carry out blunt dissection with a thumb or small scissors down between the MTs
Place a retractor or a small lamina spreader into the wound between the metatarsal heads,
and open it, placing the transverse metatarsal ligament under tension
Use a Freer elevator to bluntly dissect in line with the incision to identify the transverse
metatarsal ligament and the normal interdigital nerve proximally. The nerve occasionally
courses close to the metatarsal and is not always in the center of the interspace .
Plantar pressure under the web space should reveal the enlarged neuroma protruding distal to
the transverse metatarsal ligament . Then sharply divide the metatarsal ligament.
Identify the nerve in the proximal part of the wound and trace it distally into the irregular
mass. Look for any accessory branches coming from either metatarsal to join the common
digital nerve. If one is identified, dissect it and clearly define it. This prevents the nerve end
from retracting & forming a potential painful neuroma stump directly under metatarsal head .
Trace the common digital n. and transect it in the most proximal aspect of the wound so the
cut end does not end up bet metatarsal heads. Then dissect neuroma out distally and excise
PLANTAR APPROACH
Palpate the metatarsal heads on each side of the involved interspace and draw them
Place a small-gauge needle (25-27 gauge) from a dorsal to plantar direction in the interspace,
piercing the plantar aspect of the foot. This identifies the interspace plantarward so the
incision can be made accurately. Draw the incision in line interspace, but proximal
Carry the incision through the subcutaneous tissue. Place a Weitlander retractor. Carryout
dissection with a blunt scissors in line with the skin incision. Retract slips of the plantar
fascia, exposing the interdigital nerve and neuroma. Transect. the nerve proximally and
continue dissection distal to the neuroma. Excise the neuroma as in thedorsal approach
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Age
Site
Adolescent
Principle cell
spindle cells
(ycytoplasm)
Embryonal type but occur in UT beneath epithelium
Adolescent
Limb & head
round cells
(zcytoplasm)
Adult
UL & LL
Spindle cells
y Myxoid areas
Rhabdomyoblasts
( striations)
Racquet cells
110
Synovial Tumours
A. Benign tumors
l. Tenosynovial giant cell tumor
a. Localized tenosynovial giant cell tumor
b. Diffuse tenosynovial GCT (extraarticular PVN synovitis, florid tenosynovitis)
2. Ganglion
3. Synovial chondromatosis
4. Tumoral calcinosis
B. Malignant tumors Synovial sarcoma
1. Biphasic (fibrous and epithelial) synovial sarcoma
2. Monophasic (fibrous or epithelial) synovial sarcoma
Ganglion Cyst
Ganglia are the most common cause of focal masses in the hand and characteristically arise either
from synovium of joints or tendon sheaths or from tendons, where they may cause trigger fingers.
Incidence:
Ganglion cysts are the most common soft tissue tumors of the hand and wrist.
2ND & 4TH decade ...................................... 70% (Any age, including children)
: ............................................................. 3:1
No relation to side or occupation
Etiology theories
1- MUCOID DEGENERATION:
The most accepted explanation ............ MUCOID DEGENERATION of collagen and CT.
Site:
Dorsal wrist ganglions ................... 70% (scapho-lunate lig bet 2nd & 4th ext tendon comp)
Volar wrist ganglions ................. 20% (scapho-trapezial joint just radial to FCR tendon)
Flexor tendon sheath ..................... 10% (at the level of the A1 pulley)
Gross:
Well CIRCUMSCRIBED, SMOOTH-walled, WHITE and TRANSLUCENT mucin filled cysts
Ganglion cysts may be single or multilobulated (communicate via duct network)
Ganglions are usually CONNECTED by a stalk to an underlying joint capsule or ligament.
Ultrastructure:
Highly viscous mucin................. HYALURONIC + ALBUMIN + GLOBULIN + GLUCOSAMINE
Wall is made up of ..................... COLLAGEN out epithelium, synovium, nor necrosis
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Clinical Picture: ........................................... ASYMPTOMATIC
in OA of DIP & may lead to longitudinal grooving of the nail plate long pressure on the
germinal matrix
OCCULT DORSAL GANGLION:
SCAPHOLUNATE tenderness extreme wrist motion, especially in extension. Radiographic findings
are often normal, and MRI is useful in confirming the diagnosis. Surgical excision of the occult
ganglion is successful for alleviating pain and symptoms in the majority of cases.
Investigations
PXR (cyst is not seen) ............................... evaluate any bone or joint abnormality
MRI ............................................................ confirm atypical presentation e.g. occult ganglia
ALLEN TEST ................................................. evaluate the blood flow to the hand
TREATMENT
Aspiration steroid injection. This is especially successful for tendon sheath ganglions in the hand
and digits. Avoid multiple steroid injections to avoid skin and fat atrophy & hypopigmentation.
SURGICAL THERAPY:
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xanthomatous non neoplastic lesion affecting synovium of joints and tendons, characterized by
deposition of hemosidrin and cholesterol. It occurs due to idiopathic synovitis.
Epidemiology
2/106/y
Age ......................................................... 20-45 y (range 11-70)
=........................................................ equal
No environmental, genetic, ethnic or occupational predilection .
Since bursae & tendon sheaths are related to synovium, they may have xanthomatous growths
Commonest presentation ....................... monoarticular pain and swelling .
Sometimes .............................................. polyarticular involvement
Pathogensis Theories:
1.
2.
3.
hemosiderin are often a prominent feature, all of which factors are caused by extravasated
blood. But the lesion is not common in hemophilics who commonly bleed into their joints &
also the lesion can't be produced by injecting blood into the joint cavity.
4. Recently; ALLERGIC INFLAMMATORY REACTION to hemosiderin or its metabolites
Pathology:
SITE:
113
MICROSCOPICALLY:
1. IN THE EARLY STAGE:
Fibrosis
Pigment
++
Cholesterol
++
++
Diagnos
osis:
Vascularity
++
++
Two types:
PVN
1- Diffuse
2- Localized
Age: .... 20 in diffuse
............ 30 in localized
Male & knee are the most common
Exam:
1- Large BOGGY swelling................ in diffuse form
As
piration:
sp
114
Discrete mass(s) fixed to the tendons of the hands & feet & Achilles (and move them)
At first they are PAINFUL .............. later they are PAINLESS
Spontaneous TENDON RUPTURE IS RARE
BILATERAL involvement is usual
DISCRETE, SOFT TO FIRM, PEA-SIZED
Some times ................................. large, bulbous & disfiguraing & they are fixed to &
Overlying skin............................. normal & freely movable over the tumours
Growth is very slow .................... Take years
Yellow plaques around the eye... Part of familial hypercholesterolemic xanthomatosis
Lab:
PXR:
Pressure erosion of the adjacent phalanx. The articular surfaces are never involved.
Treatment:
1. PVN
Localized form ............................ MARGINAL EXCISION
Diffuse form ................................ TOTAL SYNOVECTOMY Radiotherapy if surgery fail
If significant bony erosion .......... ARTHROPLASTIC RECONSTRUCTION
2. Giant cell tumour of tendon sheath:
EXCISION, which may be technically difficult in large lesions
Recurrence
115
Tumoral Calcinosis
Definition:
Epidemiology
Children
Once occurred other lesions are common to follow!!
Pathogenesis Theories:
Resume
-Child
-Shoulder swelling
-Encapsulated cyst
-Ca salts
-Amorphous & crystals
-MnGC + lymphocytes
Pathology:
SITE:
MACROSCOPICALLY:
MICROSCOPICALLY:
Clinically:
DD:
1- Calcinosis universalis:
Generalized calcification of skin, s.c. tissues & muscles in children.
2- Calcinosis circumscripta:
Localized deposits of Ca associated with Raynaud's or scleroderma in adults.
Treatment:
If a solitary lesion of tumoral calcinosis is discovered one can expect that other lesions will
develop in other joints as the patient ages
They are quite apt to recur
116
Synovial Chondromatosis
SYNOVIAL CHONDROMETAPLASIA
Definition:
RARE lesion resulting from cartilaginous or osteocartilaginous
Chondromatosis
Pathogenesis Theories:
Pathology:
SITE:
KNEE, elbow, ankle, hip & shoulder are involved in this order of frequency.
The condition is usually........................ MONOARTICULAR but both knees may be affected.
MACROSCOPICALLY:
HUNDREDS OF SPHEROID CARTILAGINOUS NODULES protrude into the joint cavity
JOINT MICE or RICE BODIES may be formed
Arthroscopically it has a characteristic SNOW STORM appearance
MICROSCOPICALLY:
117
Tenosynovial chondrometap1asia:
Sometimes the tendon sheath or bursal synovia may assume the same picture
DDX: .............................................................. CHONDROSARCOMA.
Cartilage SIZES VARY ...............................from microscopic to over a centimeter in diameter.
As they age ............................................their CENTERS MINERALIZE radiopaque.
Sometimes they .................................... LIE in the sheath wall
Other times are found............................as FREE BODIES in the sheath lumen
WRITS & hand.........................................most involved in tenosynovial chondrometaplsia
PXR:
Monoarticular swelling
Rheumatic arthritis ............................... fragments of articular cartilage detach & incorporate
in the synovium or become free in the joint
Severe OA as multiple osteophytes........ may detach
Treatment:
TOTAL SYNOVECTOMY + REMOVAL OF LOOSE BODIES
118
Definiittion:
Epidemiology
10% of sarcomas
Age:....................................................... 32y
: ....................................................... 3:2
Pathogenesis Theories:
Pathology:
SITE:
MACROSCOPICALLY:
MICROSCOPICALLY:
Incidence
Slits
Cells
Stroma
Commonest
++
+
-
Spindle
Spindle & epithelioid
Pleomorphic & round
++
Pseudoacinar
-
Least
Diagnosis:
Clinically:
Slowly growing over years before seeking an advice & misdiagnosed as benign (small size)
Usually male 32y a painful swelling below the mid-thigh
as it turns severely painful
1- Hyperintense fluid level
Soft to firm, nodular, tender
PXR:
Treatment:
AMPUTATION
119
Fibrous Benign
1. Calcifying aponeurotic fibroma
Slow Growing, painless mass ,ages 3-30
Xrays show faint mass with stippling
Histology shows fibrous tumour with some calcification and cartilage formation
50 % recurrence after excision
Resolves with maturity
2. Nodular fasciitis
Painful reactive rapidly enlarging lesion in a young person
50% in upper limbs
Histology- short irregular bundles and fascicles, only small amounts of mature collagen
Treat with excision with marginal resection
3. Palmar (Dupuytrens) and plantar (Ledderhosen) fibromatoses
4. Extraabdominal Desmoid tumour
Most locally invasive of the benign soft tissue tumours
Most common in adolescents and young adults
Rock hard on palpation
May be multiple lesions
Histologically well differentiated fibroblasts and abundant collagen
Infiltrates surrounding tissues
Surgery aims at excision with a wide margin
Local recurrence common
Fibrous Malignant
1. Fibrosarcoma
Enlarging painless mass
Age group 30-80
Usually 10cm in size before symptoms
Plain Xray usually normal unless encroaching on bone
MRI deepseated inhomogenous mass
Histology- fasciculated growth pattern with fusiform or spindle shaped cells, scanty
cytoplasm, indistinct borders separated by interwoven collagen, or herringbbone appearance
Treatment- Wide local excision. If > 5cm add radiation therapy, preop/postop/periop
2. Fibrohistiocytic
Dermatofibrosarcoma protruberans
Rare, nodular cutaneous tumour
Occurs in early adult life
Intermediate in grade
Recurs locally but only rarely metastasises
Treat with wide resection
3. Malignant fibrohistiocytoma
Similar to malignant fibrosarcoma
Histology slightly different with cartwheel pattern to the spindle and histiocytic cells
Treat as for fibrosarcoma
120
121
122
Bone Metastases
Bone is the third most common site of metastatic disease
Cancers most likely to metastasise to bone:
1. Breast
2. Lung
3. Prostate
4. Thyroid
5. Kidney
6. Bowel
Carcinomas are much more likely to metastasise to bone than sarcomas
The axial skeleton is seeded more than the appendicular skeleton, partly due to the persistence
of red bone marrow in the former
The ribs, pelvis and spine are usually first affected
Batson's vertebral venous plexus allows cells to enter the vertebral circulation without first
passing through the lungs. The sluggish blood flow in this plexus is more conducive to tumour
survival, accounting for the high rate of prostate cancer metastasis to the spine.
CLINICALLY:
Pain, pathological fractures and hypercalcemia are the major sources of morbidity with bone
metastasis
Pain is the most common symptom found in 70% of patients with bone metastases. Pain is
caused by stretching of the periosteum by the tumour as well as nerve stimulation in the
endosteum
Pathological fractures are most common in breast cancer due to the lytic nature of the lesions.
They are uncommon in lung cancer due to short life span and rare in prostate cancer which
tend to be osteoblastic lesions
PXR
Lytic bone metastases must be greater than 1 cm and have destroyed 30-50% of the bone
density in order to be seen by x-ray
Metastatic bone lesions can be described as osteolytic, osteoblastic and mixed
The osteolytic lesions are most common where the destructive processes outstrip the laying
down of new bone
Osteoblastic lesions result from new bone growth that is stimulated by the tumour
WORKUP
Known Primary:
Bone Scan - Galasko reported that a period of two to eighteen months was necessary before a
lesion identified on bone scanning could be visualised on plain x-rays
FBC, Bone Biochemistry, LFT, Coagulation screen, G&S
Biopsy not necessary
Unknown Primary:
FBC, Bone Biochemistry, LFT, Coagulation screen, G&S
SPEP, TFT, PSA, Tumour markers, ESR, CRP
Bone Scan
CXR
CT chest & abdomen (or USS)
Biopsy lesion(s) for histology & microbiology (incl. TB)
123
TREATMENT
NORMALLY PALLIATIVE
RADIOTHERAPY:
o
o
o
o
o
BIPHOSPHONATES
o
o
o
o
o
o
PATHOL
OLOGICAL FRACTURE:
Pathological fractures have been reported to occur in 9-29% of patients who have bone
metastases, depending on the location of the lesion
High risk of pathological fracture =
1. > 50% loss of the cortex / shaft diameter on any view
2. Avulsion of the lesser trochanter is an indication of imminent hip fracture
3. (>2.5cm lesion)
4. MIRELS SCORING SYSTEM (CORR 1989) - a numerical score according to 4 variables:
location of the lesion
PXR appearance
the degree of pain
the size of the lesion
Mirels recommended: < 7 irradiation; > 7 operative treatment
Goals of surgery:
1. relief of pain
2. restoration of the ability to walk
3. preserve stability and function
4. an increased duration of survival
5. improved fracture-healing
Options:
o IM Nails + DXT entire bone
o Prosthesis - Epiphyseal fractures
o Endoprosthesis 4 extensive lesions
o major bone defects use PMMA
Emergency surgery is done for spinal metastasis in the hope of preserving neurological
function.
Postop radiotherapy should be considered in all cases, once wound healing has occurred.