The clinicopathologic spectrum of putative extrarenal rhabdoid tumors: An analysis of 42 cases studied with immunohistochemistry or electron microscopy

D. M. Parham, D. A. Weeks, J. B. Beckwith

Research output: Contribution to journalArticle

291 Scopus citations

Abstract

The existence of extrarenal rhabdoid tumor (ERRT) as a discrete pathologic entity has been controversial despite frequent reports of its occurrence. We performed immunohistochemistry, electron microscopy, or both on 42 cases with this diagnosis sent in consultation to us. Only 12 of the 42 neoplasms had the histological findings of 'classic' malignant rhabdoid tumor of the kidney; the remainder displayed a variety of neural, epithelial, myoid, mesenchymal, or ependymal patterns. Electron microscopy also showed that most possessed neural, epithelial, or ependymal features. Immunohistochemistry generally revealed marked polyphenotypia, with immunoreactivity to a wide array of antibodies against neural, epithelial, glial, and myogenic markers. A specific tissue-based diagnostic category could not be assigned in only 11 of the 42 cases, seven of which lacked material for a comprehensive ultrastructural or immunohistochemical study. We conclude that tumors currently diagnosed as ERRT represent a heterogeneous group of neoplasms that may form unique subsets of known entities within the specific site where they arise or that may defy classification into a specific alternative category. Our findings lead us to believe that the term ERRT is not valid as representing a specific diagnostic entity and to prefer the term 'poorly differentiated neoplasm with rhabdoid features' for undifferentiated tumors.

Original languageEnglish (US)
Pages (from-to)1010-1029
Number of pages20
JournalAmerican Journal of Surgical Pathology
Volume18
Issue number10
DOIs
StatePublished - 1994

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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