BONE TUMOURS

FIBROUS TUMOURS
Prof. R. N .Srivastav
Dr. Anil Panda
FIBROUS MEANING
 fibrous tissue. Type:Term. Definitions. 1. a tissue
composed of bundles of collagenous white fibers
between which are rows of connective tissue cells;
the tendons, ligaments, aponeuroses, and
some of the membranes, such as the dura mater.
 Non ossifying fibroma
 Fibrous cortical defect

 Fibromatosis

 Fibroxanthoma

 Metaphyseal fibrous defect

 Non osteogenic fibroma

 Fibrous dysplasia
NON OSSIFYING FIBROMA
metaphyseal fibrous defect
nonosteogenic fibroma
cortical desmoid
fibrous cortical defect
fibromatosis
Fibroxanthoma

MC benign tumor in 5-15 yrs

 Locations-----metaphysis
 80% in lower extremity
 distal femur > proximal tibia > proximal fibula > distal
tibia
 uncommon in proximal femur, proximal humerus
 Pathophysiology
abnormal osteoclastic resorption at the subperiosteal
level during remodeling of the metaphysis

 Associated conditions
 Jaffe-Campanacci syndrome
multiple NOF and
 cafe au lait , mental retardation,heart, eyes, gonads
involved
 neurofibromatosis
 ABC
 Presentation

 asymptomatic and found incidentally

 painless
 may present with pathologic fracture

 Radiographs
 diagnostic
 metaphyseal eccentric "bubbly" lytic lesion surrounded
by sclerotic rim
 cortex may be expanded and thin
 as bone grows
 migrates to diaphysis
 lesions enlarge (1-7cm)
 lesions become sclerotic as patient approaches skeletal
maturity

 CT
predicting fracture risk
Differential
 Giant cell tumor
 painful
 rare in skeletally immature
 no mineralization

 Osteosarcoma
 painful
 irregular zone of bony destruction with less defined
zone of transition
 periosteal reaction
 mineralized soft tissue mass
 TREATMENT
•Nonoperative
• observation

• most lesions resolve spontaneously and progressively reossify

as child enters 2nd and 3rd decade of life
• radiographs at 6, 12months, then annually until reossified
• casting

• pathologic fracture

•Operative
• curettage and bone grafting
• indication
• symptomatic and large lesion
• increased risk of fracture shown on quantitative CT
FIBROUS CORTICAL DEFECT
 Size ----------- <3 cm
 NO involvement of medullary cavity
FIBROUS DYSPLASIA
 benign, nonfamilial disorder
 characterized by the presence of expanding
intramedullary fibro-osseous tissue in one or
more bones.

TYPES

1. Monostotic
2. Polyostotic
3. Mc Cune Albright Syndrome
 Age: 3-30.
 Sex: F > M.
NATURAL HISTORY
 Polystotic FDis more aggressive than
monostotic FD.
 The lesions usually progress in number till the
end
 of skeletal maturation, by then they become

 quiescent

 in only about 5% of cases it continue to enlarge

 after that.
LOCATION :
 Long bones specially the neck of femur
 Skull (including calvarium, skull base, facial
bones
 & mandible),

 Pelvis
 Ribs (fibrous dysplasia is the most common
cause
 of rib expansion).

 Distribution:

 Unilateral or rarely bilateral asymmetrical.

LONG BONE FIBROUS DYSPLASIA
Meta-diaphyseal. The epiphysis is usually spared.
Central , expansile
Cortex thinned with no cortical breach or
periosteal reaction
 Density:
depend upon the ratio between the fibrous & osseous
tissues,
Sclerotic (increased osseous content), or
Lytic (increased fibrous content) or
Ground glass, (relatively equal ratios between fibrous &
osseous content).
 Margin:
Well defined sclerotic margin (Geographic bone
destruction).
The sclerotic margin may be thick (rind sign).
PELVIS FIBROUS DYSPLASIA
 Expansile, lytic,
bubbly lesion.
 The degree of
expansion & the
bubbly nature
 are quite marked in
the pelvis rather than
long
 bones.
RIB FIBROUS DYSPLASIA
 Expansile lytic lesion
with no rib
destruction
SPECIAL FORMS OF FIBROUS DYSPLASIA
 Leontiasis ossea.
 Cherubism.
 Mc Cune Albright
syndrome.
LEONTIASIS OSSEA
 A special form of
polystotic fibrous
dysplasia that
 affects the skull &
facial bones
CHERUBISM

Familial fibrous dysplasia of

the jaws
MC CUNE ALBRIGHT
SYNDROME

 Almost exclusively
affect females.
MC CUNE ALBRIGHT SYNDROME
 Never cross the
midline
 Irregular borders

 (coast of Maine)
NEUROFIBROMATOSIS
 Smooth borders
 (coast of California)

 Cross the midline.

SHEPHERD CROOK DEFORMITY
GROUND GLASS APPEARANCE
COMPLICATIONS OF FIBROUS DYSPLASIA
 1. Pathological fracture.
 2. Bone deformity.

 3. Massive cartilage hyperplasia.

 4. Accelerated bone growth.

 5. Sarcomatous degeneration.
SARCOMATOUS DEGENERATION
 very rare, it may occur
spontaneously or
following radiation
therapy.

Cortical destruction.
Extraosseous soft
tissue component.
DIFFERENTIAL DIAGNOSIS
 SBC
 Enchondroma

 Eosinophilic
granuloma
 Hyperparathyroidism

 GCT

 Neurofibromatosis
TREATMENT

 Observation only in cases of monoostotic

asymptomatic lesions

Bisphosphonates have been used to

1.relieve bone pain
2.improve cortical thickness
3.ossification of the lesion
4.prevent pathological fracture
Surgery indications
1.progressive deformity
2.large painful lesions
3.non union

CURETTAGE WITH BONE GRAFTING

 Cancellous grafts are resorbed earlier; so cortical
strut grafts should be used wherever possible

 Deformity correction – various osteotomy

High failure rates in :

1. younger age group
2.polyostotic
3.femoral lesions
THANK YOU ALL FOR YOUR PATIENCE