Fibrous Tumours

Tumor

Age

Location

Clinical presentation

Pathology

Radiology

Treatment

Prognosis

DD

Fibrous Cortical defect (Non-ossifying fibroma)	Children, M>F	metaphyseal	Usually an incidental finding in children. Most heal spontaneously.Larger ones may -> pathological fracture (common presentation)	whorled fibrous tissue, foam cells.occasionally, small elongated giant cells Jaffe-Campanacci syndrome: multiple FCDs., cafe-au-lait spots,mental retardation,hypogonadism, ocular & cardiovascular abnormalities.	Lucent. in the cortex of a long bone.metaphyseal eccentric.Margin well defined, sometimes scalloped and often sclerosed	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		malignant fibrous histiocytoma eosinophilic granuloma osteosarcoma histiocytic lymphoma pyogenic osteomyelitis
Fibrous dysplasia	children adolescents, onset 8 yrs. M > F. (Albrights - F> M)	Ribs commonest (40%) Lower limbs more than upper limbs	Relatively common and usually monostotic.Craniofacial -> skull deformity. Epiphyses usually spared.McCune - Albrights Syndrome- Polyostotic disease, pain, fracture (85%), deformity (unilateral usually),Skin pigmentation-cafe au lait spots with serrated borders (called "coast of Maine") that tend to stop abruptly at the midline of the body .Precocious puberty (endocrinopathy). usually presents earlier, may be unilateral or widespread, affecting long bones, hands, feet & pelvis.Malignant transformation (chondrosarcoma or osteosarcoma) is about 4 %;	Bone replaced by firm, whitish tissue of gritty consistency.Vascular tumour with poorly orientated bone trabeculae separated by fibrous tissue. Bone is woven rather than lamellar . lack of osteoblastic rimming of trabeculae	Lucent lesion in medullary space. Sclerotic margin with no discernable matrix.Ground glass appearance typical. No periosteal reaction Shepherds crook deformity of proximal femur.Variable appearance with expansion of cortex	Monostotic -> curettage and grafting if symptomatic. Polyostotic -> symptomatic treatment.May require osteotomy for deformity or lengthening / shortening procedures	Monostotic lesions cease activity at puberty but may be reactivated by pregnancy.Polyostotic - 85% -> pathological fracture.malignant change occurs after radiotherapy	Pagets disease/ FCD Hyperparathyroidism osteoblastoma osteosarcoma
Malignant Fibrous Histiocytoma	>30yrs. M > F	metaphyseal knee 40%	pain, swelling.15%pathological fracture. May arise in Pagets, fibrous dysplasia, long standing osteomyelitis or irradiated bone. Mets to the lung, and other bones via the blood	Discrete greyish white rubbery tumour. Irregular bony margins.Composed of a fibroblasts, myofibroblasts & large plump foamy histiocytes fibrous component is in a "storeiform pattern" (radiating).Cell of origin- histiocytic cell/ fibroblasts/multipotent mesenchymal cell .Classification-- Myxoid/ Non Myxoid	bone often mottled or moth eaten with extension into soft tissue.Osteolytic lesion may be surrounded by reactive bone.Destructive appearance radiologically.Usually little periosteal reaction	Radical excision / Amputation or radiotherapy if not amenable to resection. reports of increased survival with neo adjuvant chemotherapy (doxorubicin, vincristine, methotrexate, T10)	Better in young.If initial procedure a wide or radical excision = more than 80% 4yr survival	mets
FIBROSARCOMA			25% have metastasised at presentation		Osteolytic lesion.Margins can range from well-defined to ragged and moth-eaten.Periosteal reaction is seen with cortical destruction Extension into the soft tissue is common	Stage 1A - limb salvaging excision with wide margin.Stage 2B - radical or wide margins with adjuvant chemotherapy or radiation therapy Classically considered radio-resistant. useful as adjuvant to surgery and chemotherapy. irradiation of lung secondary deposits not accessible to surgery	Stage 2 fibrosarcoma is guarded	Mets multiple myeloma MFH leiomyosarcoma