Primary intra-osseous myoepithelioma of phalanx mimicking an enchondroma.

Myoepitheliomas (MEs) are uncommon tumours of the soft tissue with an intermediate biological behaviour and uncertain differentiation. Primary intra-osseous MEs are rare and occur predominantly in the axial and proximal appendicular skeleton in middle-aged patients. The morphological variation of the tumour cells and stromal metaplasia may cause considerable diagnostic confusion, especially when it occurs in an unusual location. A wide panel of immunohistochemical markers is required to exclude other histological mimics. A 37-year-old male presented with a recurrent swelling in the right middle finger for 1-month duration. Radiographic images showed an expansile, lytic, intra-osseous lesion with high signal intensity on T2W fat-suppressed MR images in the proximal phalanx of the right middle finger without cortical breach, highly suggestive of an enchondroma. Histopathology revealed a lobulated tumour comprising of polygonal to spindle cells in groups and cords in a chondromyxoid stroma. No cellular atypia was noted. The tumour cells were immunopositive for epithelial membrane antigen (EMA), p63, S100 and smooth muscle actin (SMA), compatible with the diagnosis of an intraosseous ME. The proximal phalanx of the right middle finger was excised, revealing a similar tumour, and the patient has been on regular follow-up for the last 18 months without any recurrence. Primary intra-osseous MEs are extremely rare, and this is the second reported occurrence in small bones. A differential diagnosis of ME should be kept for enchondroma-like lesions of the bone for proper histopathological assessment and accurate diagnosis. Documentation of such cases and follow-up will enhance our understanding of their clinical course and prognosis.