An 17 year old boy presenting with swelling in lower thigh which was progressively increasing in size, having pain since 10 days.
Discussion:
OSTEOCHONDROMA (EXOSTOSIS) Definition: - Cartilage capped bony projection arising on the external surface of bone containing a marrow cavity that is continuous with that of the underlying bone. Epidemiology: - Most common bone tumor. - Osteochondroma may be solitary or multiple, the latter occuring in the setting of hereditary multiple exostoses. - Solitary lesions account for 80% of cases, and most affected patients are diagnosed in their second decade of life - Male preponderance with a male to female ratio 1.5-2:1. - Hereditary multiple exostoses (HME) is an autosomal dominant genetic disorder , and has prevalence of 1 per 50 000 in the general population making it one of the more common inherited skeletal disease. - Patients with HME come to medical attention at the younger age , usually during first decade, because they cause severe skeletal deformities and are frequently polyostic. Sites of involvement: - Generally arise in bones performed by cartilage. - Most common site of involvement is the metaphyseal region of distal femur, uppr humerus, upper tibia and fibula. Clinical findings: - Many, if not most lesions, are asymptomatic and found incidentally. In symptomatic cases, the symptoms are often related to the size and location of the lesion. - Most common presentation is that of a hard of long- standing duration. Imaging: - Bulbous lesions on X rays, and they a narrow or broad (sessile) osseous radiosense stalk, which is attached to the underlying bone. - The characteristic feature is a projection of the cortex in continuity with the underlying bone. - Excessive cartilage type flocullent calcification should raise the suspicion of malignant transformation. - CT scan or MRI images typically show continuity of the marrow space into the lesion. A thick cartilagenous cap rises suspicion of malignant transformation.Gross: - May be sessile or pedunculated. - The cortex and medullary cavity extends into the lesion. - The cartilage cap is usually thin. - A thick an irregular cap (greater than 2 cm) may be indicative of malignant transformation. Histopathology: - Lesion has three layers - perichondrium (fibrous layer covering cartilage), cartilage and bone. - Outer layer is a fibrous perichondrium that is continuous with the periosteum of the underlying bone. - Below this is a cartilage cap that is usually less than 2 cm thick (and decreases with age). - Within the cartilage cap the superficial chondrocytes are clustered, whereas the ones close to bone resemble growth plate. - Loss of the architecture of cartilage, widefibrous bands, myxoid change, increased chondrocyte cellularity, mitotic. activity, significant chondrocyte atypia and necrosis are all features that may indicate secondary malignant transformation. Genetics: - Abberations involving 8q22-24.1, EXT1 gene. Prognosis: - Excision is usually curative. - Recurrence is seen with incomplete removal.
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