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成人横纹肌肉瘤

Rhabdomyosarcoma in adults.

作者信息

Hollowood K, Fletcher C D

机构信息

Department of Histopathology, St Thomas's Hospital (U.M.D.S.), London, England.

出版信息

Semin Diagn Pathol. 1994 Feb;11(1):47-57.

PMID:8202646
Abstract

Rhabdomyosarcomas occur in adults in one of two forms; as sporadic cases of the juvenile histological types and more commonly, if controversially, as the major adult form, pleomorphic rhabdomyosarcoma. This review documents the historical background to the fluctuating diagnostic popularity of pleomorphic rhabdomyosarcoma, together with a presentation of its defining clinicopathologic characteristics, and presents the clinicopathologic features of the juvenile types as they occur in adults. The concept of pleomorphic rhabdomyosarcoma as a distinct clinicopathologic entity, presenting as an aggressive, predominantly spindle-cell tumor arising in the skeletal musculature of middle-aged adults and defined by the presence of large, pleomorphic tumor cells, which show, at least focally, immunophenotypic or ultrastructural sarcomeric muscle differentiation, is discussed and supported.

摘要

横纹肌肉瘤在成人中以两种形式之一出现

作为青少年组织学类型的散发病例,更常见的(尽管存在争议)是作为主要的成人形式,即多形性横纹肌肉瘤。本综述记录了多形性横纹肌肉瘤诊断流行度波动的历史背景,同时介绍了其明确的临床病理特征,并阐述了青少年类型在成人中出现时的临床病理特征。文中讨论并支持了多形性横纹肌肉瘤作为一种独特临床病理实体的概念,它表现为一种侵袭性的、主要为梭形细胞的肿瘤,发生于中年成人的骨骼肌,其特征是存在大的、多形性肿瘤细胞,这些细胞至少在局部显示免疫表型或超微结构的肌节肌肉分化。

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