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Overview
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The INI-1 gene, which encodes a functionally uncharacterized protein component of the hSWI/SNFchromatin remodeling complex, is often mutated or deleted in malignant rhabdoid tumor (MRT). Twoisoforms of INI-1 that differ by the variable inclusion of amino acids are potentially produced bydifferential RNA splicing.The morphology of MRTs can present challenges in differential diagnosis. The overall survival of MRTsrelative to its potential mimics [medulloblastoma, supratentorial primitive neuroectodermal tumors(sPNETs)] is quite low, and thus differentiation from these other tumors is desirable. Lack of nuclearlabeling by anti-INI-1 is characteristic of MRT. The majority of medulloblastomas and sPNETs arelabeled by anti-INI-1. MRTs also originate from the kidney and soft tissues.
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Overview