Cerebral Tumor with Rhabdoid Features

Abstract

Rhabdoid tumors have been reported to arise from various locations such as the central nervous system, soft tissues, liver, retroperitoneum, pelvis, and skin, in addition to the kidneys. These tumors with rhabdoid cell components have been reported to be aggressive lesions. Atypical teratoid/rhabdoid tumors (AT/RT) are the most common cerebral tumors with rhabdoid features. Recently, new disease categories, such as rhabdoid meningioma and rhabdoid glioblastoma, have been reported. These tumors are difficult to distinguish radiologically. Histological and immunohistological examinations are useful for making an accurate diagnosis. Recent investigations have revealed that the lack of INI-1 gene expression is the most characteristic feature in AT/RT and malignant rhabdoid tumor. We recently reported a frontal brain tumor with rhabdoid features that could not be categorized in any of the current classifications, and referred to this tumor as a cerebral tumor with extensive rhabdoid features. We present several types of cerebral tumors with rhabdoid features and discuss the differences among them.