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一例具有肌源性和神经源性成分的胸肺区域小圆细胞肿瘤。

A case of small round cell tumor of the thoracopulmonary region with myogenic and neurogenic elements.

作者信息

Goji J, Sano K, Murakami R, Nakamura H, Ninomiya M, Ito H

机构信息

Department of Pediatrics, Kobe University School of Medicine, Japan.

出版信息

Acta Paediatr Jpn. 1992 Feb;34(1):65-71. doi: 10.1111/j.1442-200x.1992.tb00927.x.

Abstract

We here report a unique case of a young boy with an intrathoracic tumor which consisted of neurogenic and myogenic elements. The initial pathological diagnosis was alveolar rhabdomyosarcoma. The tumor tissue from surgical resection was composed of three parts, each showing a different histological appearance, i.e. a monotonous small cell area, an alveolar area, and an area consisting of pleomorphic rhabdomyoblasts. The small round cells in the monotonous area were immunoreactive with the antibodies for Leu7, neuron-specific enolase (NSE), neurofilament proteins (NFP), and beta 2 microglobulin, but not with the antibody for desmin. These cells also had dense core granules. The tumor cells in the alveolar area were immunoreactive with the antibodies for Leu7 and desmin, but not with the antibody for NFP. Pleomorphic rhabdomyoblasts were immunoreactive with the antibody for desmin, but not with the antibodies for Leu7 and NFP. These findings imply that this tumor consisted of neurogenic and myogenic elements and is considered to be a special type of rhabdomyosarcoma.

摘要

我们在此报告一例独特的病例,一名患有胸腔内肿瘤的小男孩,该肿瘤由神经源性和肌源性成分组成。最初的病理诊断为肺泡状横纹肌肉瘤。手术切除的肿瘤组织由三部分组成,每部分呈现出不同的组织学外观,即一个单调的小细胞区域、一个肺泡区域和一个由多形性横纹肌母细胞组成的区域。单调区域中的小圆形细胞对Leu7、神经元特异性烯醇化酶(NSE)、神经丝蛋白(NFP)和β2微球蛋白的抗体呈免疫反应,但对结蛋白抗体无反应。这些细胞也有致密核心颗粒。肺泡区域的肿瘤细胞对Leu7和结蛋白的抗体呈免疫反应,但对NFP抗体无反应。多形性横纹肌母细胞对结蛋白抗体呈免疫反应,但对Leu7和NFP抗体无反应。这些发现表明该肿瘤由神经源性和肌源性成分组成,被认为是一种特殊类型的横纹肌肉瘤。

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