Behtash Nadereh, Karimi Zarchi Mojgan
Gynecology Oncology Department, Vali-Asr Hospital, Keshavarz Blvd,, Tehran 14194, Iran.
World J Surg Oncol. 2007 Jun 23;5:71. doi: 10.1186/1477-7819-5-71.
Dysgerminoma is the most common malignant germ cell tumor of the ovary. This malignancy can be associated with pure gonadal dysgenesis or Swyer syndrome, mixed gonadal dysgenesis and partial gonadal dysgenesis.
Dysgerminoma developed in 3 phenotypic female patients with 46 XY pure gonadal dysgenesis. All patients presented first with abdominopelvic mass. Laparatomy was done. 46 XY karyotype was made by lymphocyte culture. Then these patients underwent gonadectomy that histopathology results were streak ovaries without evidence for malignancy. Two patients received postoperative adjuvant therapy.
In Patients with Swyer syndrome the risk of dysgerminoma is high and gonadectomy is recommended. Also 5% of dysgerminomas are discovered in phenotypic female and 46 XY karyotype, thus in adolescent with dysgerminoimas and amenorrhea, karyotype should be done.
无性细胞瘤是卵巢最常见的恶性生殖细胞肿瘤。这种恶性肿瘤可与单纯性腺发育不全或斯维尔综合征、混合性性腺发育不全和部分性腺发育不全相关。
3例具有46 XY单纯性腺发育不全的表型女性患者发生了无性细胞瘤。所有患者最初均表现为腹盆腔肿块。进行了剖腹手术。通过淋巴细胞培养确定为46 XY核型。然后这些患者接受了性腺切除术，组织病理学结果显示为条索状卵巢，无恶性证据。2例患者接受了术后辅助治疗。
斯维尔综合征患者患无性细胞瘤的风险较高，建议进行性腺切除术。此外，5%的无性细胞瘤在表型女性和46 XY核型中被发现，因此对于患有无性细胞瘤和闭经的青少年，应进行核型分析。
