Abstract

Background: SMARCB1/INI-1 deficient tumours are a phenotypically diverse group of tumours, characterised by epithelioid morphology and cytokeratin positivity, and linked to several adult and paediatric sarcomas. This group includes epithelioid malignant peripheral nerve sheath tumour, epithelioid sarcoma of proximal type and extrarenal rhabdoid tumour.

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