Yada-Hashimoto Namiko, Komura Hiroko, Nagata Shigenori, Kubo Chiaki, Fujita Masami, Kamiura Shoji
a Department of Gynecologic Oncology , Osaka International Cancer Institute , Osaka , Japan.
b Koumura Women's Clinic , Osaka , Japan.
Gynecol Endocrinol. 2018 Jun;34(6):464-466. doi: 10.1080/09513590.2017.1395844. Epub 2017 Oct 31.
Patients with Swyer syndrome, which is also known as 46,XY pure gonadal dysgenesis, are at an increased risk of gonadoblastoma and germ cell tumor. Prophylactic gonadectomy is recommended for these patients. We report a case of stage IIA dysgerminoma arising in a streak gonad in a patient with Swyer syndrome, which was not diagnosable preoperatively and intraoperatively. The patient was primarily amenorrheic and identified as female phenotypically. She underwent gonadectomy at 27 years of age. Preoperative image analysis showed a relatively small uterus without adnexal masses. Laparoscopic findings showed bilateral streak gonads. Postoperatively, histopathological examination revealed that the patient had dysgerminoma in her left streak gonad. Preoperative and intraoperative diagnosis of dysgerminoma in normal size ovaries is thought to be difficult. Although it is rare, considering the occurrence of dysgerminoma in streak gonad with extension to the mesosalpinx, prompt prophylactic gonadectomy is strongly recommended for these patients regardless of the size of the ovaries.
Swyer综合征患者，也称为46,XY单纯性腺发育不全，患性腺母细胞瘤和生殖细胞肿瘤的风险增加。建议对这些患者进行预防性性腺切除术。我们报告一例Swyer综合征患者条索状性腺发生的IIA期无性细胞瘤病例，术前和术中均未诊断出来。该患者原发性闭经，表型为女性。她27岁时接受了性腺切除术。术前影像分析显示子宫相对较小，无附件肿块。腹腔镜检查发现双侧条索状性腺。术后组织病理学检查显示患者左侧条索状性腺存在无性细胞瘤。正常大小卵巢的无性细胞瘤术前和术中诊断被认为很困难。尽管罕见，但考虑到条索状性腺发生无性细胞瘤并延伸至输卵管系膜，强烈建议对这些患者无论卵巢大小均尽早进行预防性性腺切除术。
