Adenocarcinomas of the gastrointestinal tract with prominent rhabdoid features

Research output: Contribution to journalArticle

44 Scopus citations

Abstract

Rhabdoid tumor, first described in kidneys of infants and children, is an aggressive tumor that has been reported in several extrarenal locations. Gastrointestinal tumors with rhabdoid features are extremely rare. The effect of the rhabdoid phenotype on the aggressiveness of gastrointestinal tumors remains unclear. We present four cases of rhabdoid tumors of the gastrointestinal tract involving the esophagus, stomach, and small intestine and discuss the clinicopathologic, immunohistochemical, and ultrastructural features. In the four cases reported herein, the patients' ages ranged from 52 to 73 years, and tumor size ranged from 3.8 to 13 cm in greatest dimension. The noncohesive rhabdoid cells exhibited an eccentric nucleus with a paranuclear inclusion, which was shown by electron microscopic examination to be composed of intermediate filaments. On immunohistochemical staining, the tumor cells were positive for vimentin and cytokeratin. Three patients developed distant metastasis shortly after diagnosis and died of disease within 2 to 10 months after initial presentation. A retrospective review of outcomes of the current cases and previously published literature showed that 12 (75%) of the 16 patients died within 6 months of presentation. Recognition of the rhabdoid phenotype in gastrointestinal tract neoplasms is important because this feature is associated with poor prognosis and unresponsiveness to conventional therapy.

Original languageEnglish (US)
Pages (from-to)357-363
Number of pages7
JournalAnnals of Diagnostic Pathology
Volume6
Issue number6
DOIs
StatePublished - Jan 1 2002

Keywords

  • Esophagus
  • Extra-renal
  • Gastrointestinal
  • Rhabdoid tumor
  • Small intestine
  • Stomach

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Fingerprint Dive into the research topics of 'Adenocarcinomas of the gastrointestinal tract with prominent rhabdoid features'. Together they form a unique fingerprint.

Cite this