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Keratin‐positive fibrotic extraskeletal myxoid chondrosarcoma: a close mimic of myoepithelial tumour

Extraskeletal myxoid chondrosarcoma (EMC) is a rare form of adult sarcoma with distinct histology and NR4A3 gene fusion. Immunohistochemically, EMCs are variably positive for S100 protein and neuroendocrine markers. Unlike… Click to show full abstract

Extraskeletal myxoid chondrosarcoma (EMC) is a rare form of adult sarcoma with distinct histology and NR4A3 gene fusion. Immunohistochemically, EMCs are variably positive for S100 protein and neuroendocrine markers. Unlike histologically similar soft‐tissue myoepithelial tumours, keratin expression is rare. Prompted by two recent EMC cases with diffuse keratin expression, we investigated the expression of epithelial markers in a molecularly confirmed cohort of EMC and identified two additional similar cases.

Keywords: keratin positive; myxoid chondrosarcoma; positive fibrotic; extraskeletal myxoid

Journal Title: Histopathology
Year Published: 2023

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