Primary rhabdoid cancer of the ileum: a case report and review of the literature.

Since the first publication of a rhabdoid cancer, described as an infrequent variant of Wilms' tumor, several cases of extrarenal rhabdoid tumor have been reported in the literature. Here, we report on a primary rhabdoid cancer of the small intestine, and give a review of the data available in the literature. An 81-year-old male patient was admitted to the Department of Internal Medicine with subileus and bloody stools. While gastroscopy and colonoscopy failed to identify a neoplastic disease, abdominal US and CT raised the suspicion of a malignancy involving the descending colon. Ileus-mandated laparotomy disclosed an obstructive tumor of the ileum. This segment was resected, followed by several relapses, and the patient died after a 7-month-period of non-relenting deterioration. Histological work-up of the numerous biopsies disclosed a cellular, solid, necrotic, hemorrhagic, and invasive tumor. The cancer cells were round-to-polygonal, and scattered bizarre pleomorphic cells with prominent nucleoli were common. The overall appearance of the neoplasm was highly similar in every specimen. The immunohistochemical phenotype of the malignant cells indicated rhabdoid characteristics. A thorough search of the literature revealed additional 22 cases of primary rhabdoid GI cancers. This report aims to call the pathologist's attention to the differential diagnostic importance of this entity. Copyright 2009 Elsevier GmbH. All rights reserved.