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黏液样透明细胞肉瘤

Myxoid clear cell sarcoma.

作者信息

Kim You Chan, Vandersteen Daniel P, Jung Hong-Geun

机构信息

Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

出版信息

Am J Dermatopathol. 2005 Feb;27(1):51-5. doi: 10.1097/01.dad.0000141547.31306.4e.

Abstract

Clear cell sarcoma is a rare soft-tissue tumor presenting typically in the extremities of young adults. It has been also known as malignant melanoma of the soft parts because of the presence of melanin and cytoplasmic melanosomes. However, clear cell sarcoma is, at present, usually considered as a unique lesion because the t(12;22)(q13;q12) translocation is present only in clear cell sarcoma. Myxoid malignant melanoma is now a well-recognized morphologic variant of malignant melanoma. However, a myxoid variant of clear cell sarcoma has not been well described yet. We report a case of myxoid clear cell sarcoma occurring on the heel in a 22-year-old man. The tumor was composed of nests and fascicles of oval to fusiform cells with clear to pale eosinophilic cytoplasm, often separated by fibrous septa. The tumor cells were reactive for S-100 protein, HMB-45, and MART-1. Variably sized cysts lined by one or several layers of tumor cells were observed. Alcian blue and mucicarmine stains demonstrated prominent mucin deposition in the tumor stroma and especially in the lumen of the cysts. Fluorescence in situ hybridization for the Ewing sarcoma gene showed rearrangement in nearly all of the neoplastic cells.

摘要

透明细胞肉瘤是一种罕见的软组织肿瘤,通常发生于年轻成人的四肢。由于存在黑色素和细胞质黑素小体,它也被称为软组织恶性黑色素瘤。然而,目前透明细胞肉瘤通常被认为是一种独特的病变,因为t(12;22)(q13;q12)易位仅存在于透明细胞肉瘤中。黏液样恶性黑色素瘤现在是一种公认的恶性黑色素瘤形态学变异型。然而,透明细胞肉瘤的黏液样变异型尚未得到充分描述。我们报告一例发生在一名22岁男性足跟部的黏液样透明细胞肉瘤。肿瘤由巢状和束状排列的椭圆形至梭形细胞组成,细胞质透明至淡嗜酸性,常被纤维间隔分隔。肿瘤细胞对S-100蛋白、HMB-45和MART-1呈阳性反应。观察到由一层或几层肿瘤细胞衬覆的大小不一的囊肿。阿尔辛蓝和黏液卡红染色显示肿瘤间质尤其是囊肿腔内有明显的黏液沉积。对尤因肉瘤基因进行荧光原位杂交显示几乎所有肿瘤细胞均有重排。

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