Extrarenal rhabdoid tumors of soft tissue: Clinicopathological and molecular genetic review and distinction from other soft-tissue sarcomas with rhabdoid features

Yoshinao Oda, Masazumi Tsuneyoshi

研究成果: Contribution to journalReview article査読

110 被引用数 (Scopus)

抄録

Malignant rhabdoid tumor (MRT) of the soft tissue is a rare and highly aggressive tumor that occurs in infancy or childhood. It predominantly involves a deep axial location such as the neck or paraspinal region. Microscopically, the tumor is composed of a diffuse proliferation of rounded or polygonal cells with eccentric nuclei, prominent nucleoli and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies, arranged in sheets and nests. These characteristic 'rhabdoid cells' are also present in certain soft-tissue sarcomas such as synovial sarcoma, extraskeletal myxoid chondrosarcoma and leiomyosarcoma. The existence of rhabdoid cells in these other sarcomas is correlated with a worse prognosis for the patients. Cytogenetic and molecular analyses have shown abnormalities in the long arm of chromosome 22 and alteration of the hSNF5/INI1 (SMARCB1) gene in renal, extrarenal and intracranial MRT. This gene alteration has been considered to be a specific molecular event in MRT, but a recent study has also demonstrated frequent alteration of this gene in proximal-type epithelioid sarcoma (ES). Both MRT of soft tissue and proximal-type ES show immunoreactivity for vimentin, cytokeratin and epithelial membrane antigen. The tumor cells of proximal-type ES are also occasionally positive for CD34 and β-catenin, whereas MRT of soft tissue has no immunoreaction for these markers. Detailed clinicopathological and immunohistochemical evaluations are necessary to distinguish MRT of soft tissue from proximal-type ES, because these tumors demonstrated a similar morphology and the same gene alteration.

本文言語英語
ページ(範囲)287-295
ページ数9
ジャーナルPathology International
56
6
DOI
出版ステータス出版済み - 6 2006

All Science Journal Classification (ASJC) codes

  • 病理学および法医学

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