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Soft Tissue Tumour

Fatty Tissue Tumour

Lipoma Liposarcoma
Nature Benign Malignant
Age 40-70 40-70
Site Subcutaenous Proximal extremities
Upper back Retroperitoneum
Shoulder/ Back Paratesticular
Clinical Soft, Mobile, Painless (except angiolipoma) Painless, Deep seated
Subtypes Fibrolipoma, Angiolipoma, Myelolipoma, Spindle cell lipoma, Pleomorphic Well differentiated, Myxoid, Round cell, Pleomorphic, Dedi ferentiated
lipoma
X-ray Well-circums cribed, Homogen ous, Similar density as fat Lobulated with mass – contain strand of soft tissue slightly dense
HPE Lobules of mature adipocytes Heterogenous population of adipocytes
Fibrous septae Lipoblasts
Rx Complete resection Complete resection
Prognosis Good (except intramuscular type) Depend on location, type

Lipoma Spindle cell lipoma Liposarcoma Liposarcoma

Neoplastic adipocytes – Proliferation of adipocytes Large mass lesion Adipocytes
indistinguishable from normal Disorganized bands of collagen – Yellowish, like adipose tissue Pleomorphism of neoplastic cells
adipocytes contain spindle cells Well-differentiated (lipoblasts)

Pleomorphic lipoma Cellular angiolipoma Liposarcoma Liposarcoma

Floret cells (large pleomorphic cells Well-circums cribed tumor Large bizarre lipoblasts Well-differentiated atypical lipoma
with nuclei around cell periphery) Compose d of vascular proliferation Fibrous septa (cellular septa)
Scattered fat cells
Thrombi (in blood filled spaces)

Liposarcoma Myxoid, Liposarcoma

Well-differentiated atypical lipoma
Pleomorphic nu clei scattered among
fat cells
Branching capillary network
Small cells of myxoid liposarcoma
Lipoblasts
Lymphangioma-like cystic
degeneration

Pleomorphic, Li posarcoma
Cells with multiple grapelike vacuoles
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Smooth Muscle Tumour
Leiomyoma Leiomyosarcoma
Age 30-50 > 20
Sites Uterus Cutaneous (extremities & trunk)
Skin, nipples, scrotum, labia Large blood vessels (IVC) & deep soft tissue
Intestine, less in deep soft tissue Uterus
Signs & Painless soft tissue swelling Soft tissue swelling
Symptoms Painful in pilar leiomyoma Mass effect : obstruction
Intestinal obstruction & urinary symptoms
Size 1-2 cm (e xcept uterus) > 5 cm
HPE Circumscribed Interweaving fascicles of pleomorphic spindle cells
Bland smooth mu scle ↑ mitosis
Whorl pattern Necrosis
< 1 mitosis/ 10 HPF (high power field) Sarcomatous like MFH (malignant fibrous histiocytoma)
Degenerative changes
IM Actin + Desmin + Actin + Desmin +
Prognosis Good Depend on size, location, grade
Retroperitoneum - incomplete excision

Leiomyoma
Cells do not vary greatly in size &
Uterine fibroids/ uterine leiomyoma shape
Resemble normal smooth muscle
cells
Leiomyosarcoma
Alternating fascicle pattern
Blunt-edged nuclei
Minor pleomorphism present

Leiomyoma
Blunt-ended elongated nuclei
(smooth muscle proliferation)
Bizarre leiomyoma
Enlarged pleomorphic nuclei
Non-mitotic
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Skeletal Muscle Tumour
Rhabdomyoma Rhabdomyosarcoma (RMS)
Cardiac rhabomyoma (hemartomatous ) Common soft tissue sarcoma – children, adoles cent
1. Rare Cytogenic abnormalities
2. < 5y/o
3. Frequently associated with tuberous sclerosis Diagnostic cell = rhabdomyoblast
4. HPE – large, polygonal cells with glycogen
vacuoles
Adult rhadomyoma Sites
1. > 40 y/o 1. Head & Neck
2. Exclusively in Head & Neck region – eg. 2. Genitourinary tract
Oropharynx 3. Extremities
Genital rhabdomyoma Histological subtypes
1. 20-40 y/o Embryonal RMS Alveolar RMS Pleomorphic/ anaplastic RMS
2. Polypoidal lesion (spindle cell) at vagina, vulva,
Conventional
cervix
Botyoid (sarcoma botryoides)
Rhabdomyoma
Spindle cell
(genital type)
Infant & children Adolescent Adult
Fibrous tissue
1. Head & Neck, GUS, Extremities, paranasal sinuses, Extremities
Spindling groups of
retroperitoneum retroperitoneum
cells
2. Bladder, vagina, rectum,
Long cytoplasmic
nasal cavity, nasopharynx
extensions with
3. Paratesticular
parallel sides
Spindle cells Alveolar-like spaces Large, pleomorphic
Cross-striations Fibrous septae Multinucleated
Rhabdomyoblast Cross-striation ↑ mitosis
Primitive cells Rhabdomyoblast
Cambium layers
Abundant collagen

Rhabdomyosarcoma (Embryonal
type)
Round cells with small oval nuclei
Scanty eosinophilic cytoplasm
Eccentric placement of n ucleus in
cytoplasm
Rhabdomyosarcoma (Alveolar
type)
Tumor cells – grip fibrous septa
around alveoli
Large pleomorphic cells with
abundant cytoplasm

Rhabdomyosarcoma (Botryoid
type) Orbital conjunctiva
Dense aggregation of cells below
epithelium

Rhabdomyosarcoma (Botyroid
type)
Cambium layer
Loose texture
Differenting strap cells
IM – Vimentin, Desmin, Myogenin
Treatment – Surgery, Chemotherapy with or without radiotherapy
Prognosis – Staging, Age, Histological subtypes (Botryoid > Embryonal > Pleomorphic > Alveolar)
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Fibrous Tissue Tumour
Fibroma Fibromatosis Fibrosarcoma
Rare Superficial Deep (Desmoid tumor) Rare
Fibroma-thecoma in ovary Palmar Extra-abdominal Diagnosis by exclusion
Renal fibroma – hamartoma Plantar shoulder, chest wall, Sites
Penile back, thigh Retroperitoneum, thigh, knee, distal extremities
Abdominal Gross
anterior abdominal wall Uncapsulated, soft, infiltrative with areas of
Intra-abdominal hemorrhage & necrosis
mesentery, pelvic wall HPE
Male ↑ Female ↑ Fascicles of spindle cells
Benign Agressive locally Herringbone pattern
Do not progress Frequenty recur but Frequent mitosis & necrosis
never metastasize Prognosis
Fibroblasts Fibroblasts & collagen Aggressive
Collagen deposit Mitosis infrequent 50% recur
25% metastasize

Fibrosarcoma
Fascicles of spindle cells
Fibromatosis
Fibroblasts proliferate on collagen Lesion is cellular
Cytoplasm is inapparent, but visible if cut en face
(center)

Fibrosarcoma
Herringbone pattern
Differential diagnosis – synovial sarcoma, malignant
peripheral nerve sheath tumor
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Fibrohistiocytic tumour
BFH (Benign fibrohistiocytic tumour) DFP (Dermatofibrosarcoma protuberans) MFH (Malignant fibrohistiocytic tumour)
Benign Borderline Malignant
Dermatofibroma Common pathway for ST sarcoma progression
Age 20-50 20-50 > 50
Sex Male ↑ Male = Female Male ↑
Sites Skin Trunk Proximal extremities
Lower limb Groin Retroperitoneum
Head & Neck Lower Limb
Head & Neck
Signs & Slow growing dermal / Soft tissue (ST) mass Exophytic Large mass
Symptoms Multinodular painless growth Sometimes with pain
Gross Fairly circumscribed nod ule/ mass Poorly circumscribed 5-20 cm pse udo-capsule hemorrhage & necrosis
with entrapped skin adnexae & fat
Sub-types - Classical Storiform/ Pleo
Bednar tumour Myxoid
Inflammatory
Giant cell
Angiomatoid
Histo Spindle cells Spindle cells Depends on subtypes
Infiltrative margin Infiltrative margin
Foam cells, hemosiderin, giant cells, epidermal Prominent storiform pattern
hyperplasia Mitosis <5/10 HPF
Rx Simple excision Wide surgical resection Wide surgical resection
Prognosis Good Recurrence (50% ) Aggressive (except angiomatoid)
Metastasis (5-15%) Metastasis (30-50%)
60% - 5 year survival rate (YSR)

Malignant fibrous histiocytoma (Medium power)

Benign fibrous histiocytoma
Hyperplastic epidermis
Dermal spindle cell lesion – contain lakes of
blood
No endothelial lining of lakes
Dermatofibrosarcoma protuberans
Storiform/ pleomorphic
Cartwheel/ storiform pattern
Pleomorphic nu clei scattered among smaller cells
(without pleomorphism)
Thin nuclei, lack of perceptible cell borders, overall Vague storiform pattern
tight quality of pattern

Malignant fibrous histiocytoma (High power)

Bizarre nuclei in huge cells found
Small fibrous histiocytomas
Inflammatory infiltrate
Cartwheel/ storiform cells
Spindle cells – irregular nuclear shapes, lack well-
Lack plaquelike growth in dermis/ subcutaneous fat
defined cell borders

Malignant fibrous histiocytoma (angiomatoid) Malignant fibrous histiocytoma (inflammatory)

Dark cuff of lymphoid cells complete with Malignant fibrous histiocytoma (Giant cell)
Malignant nuclei are larger & vesicular (not bizarre)
germinal center Osteoclast-like giant cells
Sea of neutrophils & other inflammatory cells
Lighter tumour nodules proliferate Malignant tumour giant cells with enlarged nuclei
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Blood vessel tumour
Hemangioma Hemangioendothelioma Angiosarcoma
Nature Benign Borderline Malignant
Age Infant & children > 40 > 40
Sites Skin of Head & Neck ST of extremities Skin of Head & Neck
Liver Large veins Breast, liver, spleen
Skin of Head & Neck ST of extremities
HPE Benign endothelial cells Proliferating endothelial cells Malignant pleomorphic cells
In lobules Cytoplasmic vacuoles containing RBC Anastomosing blood vessels
Prognosis Good Variable Very poor
Majority cured by excision Margin clearance difficult
Up to 40% recur 60% recur
Up to 30% metastasize 50% metastasize
Capillary Cavernous Lobular capillary
Infancy, early Infant Any age
childhood Children
Female ↑ Female ↑ F=M
Skin & Skin & Gingiva, fingers,
subcutaneous of subcutaneous lip & face
Head & Neck Deep ST
Liver
Lobules of s mall Large blood- As capillary type
size vessel filled vessels Inflammation &
Central “feeder” Thrombi & ulcer
vessel calcification
Grade 2 Hemangioendothelioma Angiosarcoma
Spontaneous No spontaneous No spontaneous
Vessel-forming growth pattern
regression regression regression Collagen as scaffold for end othelial cells
Blood-filled spaces Too many cells line anastomosing spaces
Atypia

Spindle cell hemangioendothelioma

Lobular capillary hemangioma (pyogenic granuloma) Vacuolated endothelial cells
(Medium power) Lining blood-filled channels
Lobules are sharply demarcated by fibrous septa –
hypercellular & lack spindy stream of cells in Kaposi disease

Lobular capillary hemangioma (pyogenic granuloma)

(High power)
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Peripheral Nerve Tumour
Neurofibroma Schwannoma (neurilemmoma) Malignant peripheral nerve shealth tumour (MPNST)
20-40 y/o. 5-30 y/o (Diffuse) 20-50 y/o 20-50 y/o
Childhood (plexifor m) Male = Female (but Male ↑ in neurofibromatosis)
Solitary Soliary = Neurofibrosarcoma
Diffuse Multiple (3%) = Malignant schwannoma
Plexiform
Any sites (especially Head & Neck) Extremities & Head & Neck
Cellular schwannoma – mediastinum, retroperitoneal,
pelvis
Slow growing painless skin lesions Slow growing tumour Soft tissue mass with/ without pain
Plaque-like in diffuse type Pain with neurologic symptoms
Tortous, worm-like nerve Headache, nasal obstruction, epistaxis, otitis media Long sweeping fascicles & herring bone patterns
Elephantiasis neuromatosa Hearing loss + tinnitus Hypocellular myxoid areas (CT fibrosarcoma)
NF Type 1 (chromosome 17 ) NF Type 2 (chromosome 22 ) Nuclear palisading
No capsule Well-circums cribed Geographical necrosis
Fusiform, plaque, plexiform Encapsulated Metaplastic changes (eg. Bone)
Dumbbell-shape d lesion Others – rosettes, glands formation, perivascular
Spindle cell in haphazard arrangement Antoni A area condensation, neuroepithelial differentiation like
Hypocellular area Antoni B area Ewing sarcoma
Mast cells Verocay bodies Epitheloid variant (CT melanoma or carcinoma)
Hyalinized blood vessels Triton tumour (MPNST + RMS differentiation)
Cured by excision (s olitary) Cured by excision Wide surgical resection
Recur & ↑ risk malignancy (NF)

Neurofibroma Schwannoma Malignant peripheral nerve sheath tumour

Plexiform neurofibroma Dimorphic pattern of cellular (Antoni A) Mitotically active & cellular tumour
Loose myxoid (Antoni B) Nuclei bullet shaped
Nuclear palisading (parallel rows of nuclei) (blunt at one end, pointed at other)

Neurofibroma
Uniformly hypocellular
Spindle cell carcinoma
Slightly myxoid
Nervelike whorls
Undulating fibrillar background

Primitive Neuroectodermal tumour (PNET) (= Ewing sarcoma)

10-15 y/o
Swelling with pain & fever
Morphology
Small uniform round cells with scanty cytoplasm
Very little stroma
Rosette formation
Aggressive tumour
Treatment with resection & chemotherapy +/- radioRx
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Synovial Sarcoma
Carcinoma of soft tissue
20-40 y/o
Location
Lower > upper extremities & proximal > distal
Head & Neck, abdomen, retroperitoneum
Only 10% intra-articular
Radiographic findings
Deceptively well-circumscribed
Stippled calcifications
Mimic myositis ossificans (d ue to extensive calcification & metaplastic bone formation )
Pathologic findings
• Fibrous elements
o Spindle cells (monotonous, uniform) in short fasci cles
o Collagen (variable stromal)
o Alternating hypercellular & hypocellular regions
• Epithelial elements
o Gland formation
o Mucin production
• Intratumoral calcifications
• Cystic degeneration
• Metaplastic bone reaction
Subtypes
Monophasic fibrous > Biphasic > Monop hasic epithelial > Poorly differentiated
IM
Vimentin, EMA, Cytokeratin, CD99
Treatment
Surgical resection (plus adjuvant therapy)
Risk factors for disease progression
Age > 25
Size > 5
High nuclear grade (poorly differentiated)
Necrosis > 50%
Rhabdoid morp hology
↑ Tumour stage
Disease-free survival
88% in low-risk group (<25 y/o, <5cm, no poorly differentiated)
18% in high-risk group

Synovial sarcoma (Biphasic type)

Glands
Small, oval, overlapping nuclei in spindle component

Synovial sarcoma (Monophasic type) Synovial sarcoma (Monophasic type)

Pericytomatous pattern Focal cell cluster (indicate epithelial differentiation)
Solid areas

Synovial sarcoma
Synovial sarcoma (Reticulin stain)
Cytokeratin – glands, scattered spindle cells
Epithelial nesting pattern