喉肺泡软部肉瘤：一例罕见部位病例报告并进行免疫组化及超微结构分析

Alveolar soft part sarcoma of the larynx: a case report of an unusual location with immunohistochemical and ultrastructural analyses.

作者信息

Kusafuka Kimihide, Muramatsu Koji, Yabuzaki Tsugumi, Ishiki Hiroto, Asano Rie, Kamijo Tomoyuki, Iida Yoshiyuki, Ebihara Mitsuru, Onitsuka Testuro, Kameya Toru

机构信息

Pathology Division, Shizuoka Cancer Center Hospital and Research Institute, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka 411-8777, Japan.

出版信息

Head Neck. 2008 Sep;30(9):1257-63. doi: 10.1002/hed.20766.

DOI:10.1002/hed.20766

PMID:18286485

Abstract

BACKGROUND

Alveolar soft part sarcoma (ASPS) is a rare mesenchymal neoplasm of uncertain origin. In this article, we report a case of ASPS occurring in the larynx, an extremely rare location for this rather unusual tumor.

METHODS AND RESULTS

The patient was a 34-year-old Japanese woman who requested an examination for hoarseness. The tumor showed a proliferation of large polygonal cells with periodic-acid-Schiff-positive diastase-resistant intracytoplasmic granules, arranged in an alveolar growth pattern. The cytoplasm of the tumor cells was eosinophilic. Tumor cells were positive for vimentin and titin. Nuclear immunoreactivity for TFE3 was observed, and the Ki-67 labeling index was 14.7%. Ultrastructurally, electron-dense rod-shaped crystals were infrequently observed in the cytoplasm. This case was finally diagnosed as ASPS of the larynx.

CONCLUSION

We discuss the histogenesis and differential diagnosis of ASPS with immunohistochemical and ultrastructural findings. TFE3 immunohistochemistry was found to be a very useful marker for the diagnosis of ASPS.

摘要

背景

肺泡软组织肉瘤（ASPS）是一种起源不明的罕见间叶性肿瘤。在本文中，我们报告了一例发生于喉的ASPS病例，对于这种相当不常见的肿瘤来说，喉是极其罕见的发病部位。

方法与结果

患者为一名34岁的日本女性，因声音嘶哑前来就诊。肿瘤表现为大的多边形细胞增殖，具有耐淀粉酶的过碘酸希夫阳性胞浆内颗粒，呈肺泡状生长模式排列。肿瘤细胞的胞浆呈嗜酸性。肿瘤细胞波形蛋白和肌联蛋白呈阳性。观察到TFE3核免疫反应性，Ki-67标记指数为14.7%。超微结构上，在胞浆中偶尔观察到电子致密的杆状晶体。该病例最终诊断为喉ASPS。