Liang H, Li S J, Yang J X, Wu M, Cao D Y, Wang J H, Wang T, Zhang X Y
Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, National Clinical Research Center for Obstetric and Gynecologic Diseases, Beijing 100730, ChinaLiang Huan is working on the Department of Gynecology, The Central Hospital of Enshi Tujia and Miao Autonomous Prefecture, Enshi 445000, China.
Zhonghua Fu Chan Ke Za Zhi. 2024 Jan 25;59(1):64-69. doi: 10.3760/cma.j.cn112141-20230906-00088.
To evaluate the incidence, treatment, and survival outcomes of Swyer syndrome with gonadal non-dysgerminoma malignant germ cell tumor (MGCT-NDG). A retrospective study was performed on Swyer syndrome patients with MGCT-NDG between January 2011 and December 2022 in Peking Union Medical College Hospital to investigate their characteristics and outcomes. A total of 15 patients (4.9%, 15/307) with Swyer syndrome were identified in 307 MGCT-NDG patients. The average age at diagnosis of MGCT-NDG and Swyer syndrome were (16.8±6.7) and (16.7±6.6) years, respectively. Six cases were preoperatively diagnosed as Swyer syndrome, of which 4 cases received bilateral gonadectomy with or without hysterectomy, while the other 2 cases underwent removal of gonadal tumor and unilateral gonadectomy with hysterectomy, respectively. Of the 9 patients postoperatively diagnosed as Swyer syndrome, unilateral gonadectomy, removal of gonadal tumor, and unilateral gonadectomy with hysterectomy were performed in 6 patients, 2 patients, and 1 patient, respectively. Mixed malignant germ cell tumor (MGCT;10 cases), yolk sac tumor (4 cases), and immature teratoma (1 case) were the pathological subtypes, in the descending order. There were International Federation of Gynecology and Obstetrics (FIGO) stage Ⅰ in 6 cases, stage Ⅱ in 3 cases, stage Ⅲ in 5 cases, and stage Ⅳ in 1 case, respectively. Eleven patients received reoperation for residual gonadectomy after a average delay of (7.9±6.2) months, including 8 MGCT-NDG patients and 1 gonadoblastoma patient, no tumor involved was seen in the remaining gonads in the other 2 cases. Ten patients experienced at least one recurrence, with a median event free survival of 9 months (5, 30 months), of which 2 patients received surgery only at the time of initial treatment. All patients with recurrence received surgery and combined with postoperative chemotherapy. After a median follow-up of 25 months (15, 42 months), 10 patients were disease-free, 3 patients died of the tumor, 1 died of side effects of leukemia chemotherapy, and 1 survived with disease. The incidence rate of Swyer syndrome in patients with MGCT-NDG is about 4.9%; timely diagnosis and bilateral gonadectomy should be emphasized to reduce the risk of reoperation and second carcinogenesis in this population.
评估伴有性腺非生殖细胞瘤性恶性生殖细胞肿瘤（MGCT-NDG）的Swyer综合征的发病率、治疗方法及生存结局。对2011年1月至2022年12月在北京协和医院就诊的伴有MGCT-NDG的Swyer综合征患者进行回顾性研究，以调查其特征和结局。在307例MGCT-NDG患者中，共识别出15例（4.9%，15/307）Swyer综合征患者。MGCT-NDG和Swyer综合征的平均诊断年龄分别为（16.8±6.7）岁和（16.7±6.6）岁。6例术前诊断为Swyer综合征，其中4例行双侧性腺切除术，可选择或不进行子宫切除术，另外2例分别行性腺肿瘤切除术及单侧性腺切除术加子宫切除术。在9例术后诊断为Swyer综合征的患者中，分别有6例、2例和1例行单侧性腺切除术、性腺肿瘤切除术及单侧性腺切除术加子宫切除术。病理亚型依次为混合性恶性生殖细胞肿瘤（MGCT；10例）、卵黄囊瘤（4例）和未成熟畸胎瘤（1例）。国际妇产科联盟（FIGO）分期：Ⅰ期6例，Ⅱ期3例，Ⅲ期5例，Ⅳ期1例。11例患者平均延迟（7.9±6.2）个月后接受了残留性腺切除术的再次手术，其中包括8例MGCT-NDG患者和1例性腺母细胞瘤患者，另外2例剩余性腺未见肿瘤累及。10例患者至少经历了一次复发，无事件生存时间中位数为9个月（5，30个月），其中2例仅在初始治疗时接受了手术。所有复发患者均接受了手术并联合术后化疗。中位随访25个月（15，42个月）后，10例患者无疾病，3例死于肿瘤，1例死于白血病化疗的副作用，1例带瘤生存。MGCT-NDG患者中Swyer综合征的发病率约为4.9%；应强调及时诊断和双侧性腺切除术，以降低该人群再次手术和二次致癌的风险。
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