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ABSTRACT: Background
Malignant extrarenal rhabdoid tumor is a rare and aggressive soft tissue sarcoma that typically affects infants. It poses considerable diagnostic challenges owing to its overlap with other tumors showing rhabdoid features.Case presentation
A 6-year-old Indian girl presented in our institute with a gluteal mass arising from the sciatic nerve. Histopathology showed rhabdoid morphology with cytoplasmic inclusions. Immunohistochemistry revealed diffuse epithelial membrane antigen and vimentin positivity, variable cluster of differentiation 99, and loss of integrase interactor 1, consistent with malignant extrarenal rhabdoid tumor. Differential diagnoses-including epithelioid sarcoma, malignant peripheral nerve sheath tumor, chordoma, and Capicua transcriptional repre
SUBMITTER: Murhekar K
PROVIDER: S-EPMC12797898 | biostudies-literature | 2025 Dec
REPOSITORIES: biostudies-literature