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一名77岁女性的骨外尤文肉瘤。

Extraskeletal Ewing sarcoma in a 77-year-old woman.

作者信息

Cheung C C, Kandel R A, Bell R S, Mathews R E, Ghazarian D M

机构信息

Department of Laboratory Medicine and Pathobiology, University Health Network, University of Toronto, Toronto, Ontario, Canada.

出版信息

Arch Pathol Lab Med. 2001 Oct;125(10):1358-60. doi: 10.5858/2001-125-1358-EESIAY.

Abstract

Extraskeletal Ewing sarcoma (EES) is a rare soft tissue tumor that is morphologically indistinguishable from Ewing sarcoma of bone. It is usually found in young people, but several cases have occurred in patients older than 50 years. The differential diagnoses include other small, blue round cell tumors (SBRCTs) and other members of the Ewing family of tumors such as the primitive neuroectodermal tumor. We present a case of EES in the left inguinal region of a 77-year-old woman. The tumor was distinguished from other SBRCTs by lack of immunoreactivity for epithelial, lymphoid, vascular, neuroendocrine, neural, histiocytic, and muscle markers. Primitive neuroectodermal tumor was excluded because of the lack of neural differentiation by histologic analysis, immunohistochemistry, and electron microscopy. Extraskeletal Ewing sarcoma was confirmed by characteristic features on histologic analysis, histochemistry, immunohistochemistry, and electron microscopy and by the presence of the t(11;22)(q24;q12) fusion transcript detected by reverse transcriptase-polymerase chain reaction. This case serves to remind the reader that EES is not a tumor that occurs exclusively in young patients.

摘要

骨外尤文肉瘤(EES)是一种罕见的软组织肿瘤,在形态学上与骨尤文肉瘤无法区分。它通常发生在年轻人中,但也有几例发生在50岁以上的患者身上。鉴别诊断包括其他小的蓝色圆细胞肿瘤(SBRCT)以及尤文肿瘤家族的其他成员,如原始神经外胚层肿瘤。我们报告一例77岁女性左腹股沟区的EES病例。该肿瘤通过对上皮、淋巴、血管、神经内分泌、神经、组织细胞和肌肉标志物缺乏免疫反应性,与其他SBRCT相鉴别。由于组织学分析、免疫组织化学和电子显微镜检查均未发现神经分化,排除了原始神经外胚层肿瘤。通过组织学分析、组织化学、免疫组织化学和电子显微镜检查的特征性表现,以及逆转录酶-聚合酶链反应检测到的t(11;22)(q24;q12)融合转录本,确诊为骨外尤文肉瘤。该病例提醒读者,EES并非仅发生于年轻患者的肿瘤。

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