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皮下促纤维增生性小圆细胞瘤:一个不寻常的原发部位,扩大了浅表性圆细胞肿瘤的鉴别诊断范围。

Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors.

作者信息

Asadbeigi Sepideh N, Zhang Lisheng, Linos Konstantinos

机构信息

Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA.

Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA.

出版信息

J Cutan Pathol. 2020 Aug;47(8):768-775. doi: 10.1111/cup.13703. Epub 2020 Jun 2.

Abstract

Desmoplastic small round-cell tumor (DSRCT) is a rare, aggressive malignant tumor, which in the great majority of cases arises at abdominal-pelvic sites. Nevertheless, rare cases of primary extra-abdominal tumors have been reported. In challenging cases, its molecular hallmark, the EWSR1-WT1 reciprocal translocation, can be exploited diagnostically by various molecular techniques. Herein, we report an extremely rare case of primary subcutaneous DSRCT in an effort to raise awareness among our dermatopathology colleagues by expanding the differential of superficial round-cell tumors.

摘要

促纤维增生性小圆细胞肿瘤(DSRCT)是一种罕见的侵袭性恶性肿瘤,在绝大多数情况下发生于腹盆腔部位。然而,也有罕见的原发性腹外肿瘤病例报道。在具有挑战性的病例中,其分子特征——EWSR1-WT1相互易位,可通过各种分子技术用于诊断。在此,我们报告一例极其罕见的原发性皮下DSRCT病例,旨在通过扩大浅表性圆细胞肿瘤的鉴别诊断范围,提高我们皮肤病理学同行的认识。

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