Myoepithelial tumours are rare and distinct entities with uncertain histogenesis. They occur primarily in major salivary glands and soft tissue around the head and neck. Bony involvement predominantly occurs in facial bones. Their involvement in long bones is extremely rare. There is no sex predisposition with a wide age range, though they are less common in the elderly. Histologically, they show a wide morphological spectrum and cytological heterogeneity with myxoid stroma and chondroid differentiation, with cells ranging from spindled, epithelioid and plasmacytoid forms, causing significant diagnostic dilemmas. The coexpression of at least one epithelial marker and S-100 or GFAP protein is required for the diagnosis. Here we report a rare case of an early adolescent male with a myoepithelial carcinoma of the left proximal tibia, which is owing to an unusual location. This article has shared our experience, managing this rare medical identity with excellent outcomes.
Keywords: Malignant and Benign haematology; Muscle disease; Orthopaedics; Paediatric oncology.
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