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Ujjwal Bansal, Dipesh Duttaroy, Jitendra Jagtap, Gunjan Patel
Hereditary multiple exostoses (HME) or osteochondromatosis is a familial autosomal dominant disorder associated with multiple bony protrusions, with cartilage caps usually arising from the lower femur, upper tibia, upper humerus, or the pelvis. Osteochondromas are usually asymptomatic, but may be associated with cosmetic deformities, impairment of neurovascular or musculo-tendinous functions, and malignant transformation into chondrosarcoma.
We report on a patient with multiple exostoses: well-differentiated secondary chondrosarcoma arising from an iliac osteochondroma.
Case report:
A 45-year Asian Indian male presented with a huge lump on the left side of abdomen of 5 months duration with dull aching continuous pain over it for 1 month. Examination revealed bony hard projections on the medial aspect of both the lower end of femur and upper end of tibia (Figure 1C). There was a lobulated, hard, immobile retroperitoneal lump (25×20 cm) occupying the left hypochondrium, lumbar, iliac, hypogastric, and umbilical regions (Figure 1A,B) whose lower border merged with the ilium.
Figures 1A: Anterior abdomen showing the lump; 1B: Left lateral abdomen showing the lump; 1C: Anterior aspect of both knee joints showing exostosis arising from the tibia
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Ultrasonography demonstrated a retroperitoneal mass with mixed echogenicity extending into the pelvis. Radiography revealed a large soft tissue shadow with multiple calcific foci in left abdomen (Figure 2A) as well as pedunculated exostoses of tibia and left femur, and sessile exostoses of the right femur and the left fibula (Figure 2B).
Figures 2A: Radiograph of abdomen showing calcification with soft tissue shadow in the left lower abdomen; 2B: Radiograph both knees showing multiple exostoses
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Abdominal CT (Figure3 A,B,C) showed a large heterogeneously...