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一名患有斯维尔综合征的患者,其双侧性腺母细胞瘤被右侧性腺的无性细胞瘤过度生长。

Bilateral Gonadoblastoma Overgrown by Dysgerminoma of the Right Gonad in a Patient with Swyer Syndrome.

作者信息

Arafa Mohammad, Ryiami Marwa Al, Shukri Maryam Al, Burney Ikram, Mahfouz Yasser, Al-Kindi Nashwa

机构信息

Department of Pathology, Sultan Qaboos University Hospital, Oman.

Department of Obstetrics and Gynecology, Sultan Qaboos University Hospital, Oman.

出版信息

Maedica (Bucur). 2021 Dec;16(4):734-737. doi: 10.26574/maedica.2020.16.4.734.

Abstract

Patients with Swyer syndrome, "XY gonadal dysgenesis", have fibrosed gonads with a significant risk of developing germ cell tumours. During radiological assessment, a 17-year-old female with Swyer syndrome showed mildly enlarged gonads that were removed laparoscopically and proved pathologically to be bilateral gonadoblastomas. In addition, the right sided lesion showed overgrowth by dysgerminoma. The patient was further treated with combination chemotherapy and she was in complete remission for three years.

摘要

患有斯维尔综合征(“XY性腺发育不全”)的患者,其性腺会发生纤维化,并有发生生殖细胞肿瘤的重大风险。在影像学评估期间,一名患有斯维尔综合征的17岁女性显示性腺轻度肿大,通过腹腔镜手术将其切除,病理检查证实为双侧性腺母细胞瘤。此外,右侧病变显示有未成熟畸胎瘤过度生长。该患者进一步接受了联合化疗,目前已完全缓解三年。

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本文引用的文献

1
Swyer Syndrome: A Case of Dysgerminoma Solely within the Fallopian Tube.
J Pediatr Adolesc Gynecol. 2021 Dec;34(6):869-871. doi: 10.1016/j.jpag.2021.04.008. Epub 2021 May 11.
3
A Case of 45,X/46,XY Mosaicism Presenting as Swyer Syndrome.
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