Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, National Clinical Research Center for Obstetric and Gynecologic Diseases, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China.
Orphanet J Rare Dis. 2021 Nov 18;16(1):480. doi: 10.1186/s13023-021-02111-w.
Primary cervical gestational trophoblastic neoplasias (GTNs) are extremely rare ectopic GTNs. Such lesions are difficult to diagnose clinically because of their rarity, with abnormal vaginal bleeding of a non-specific cause being the most common symptom. To that end, this retrospective study aimed to identify the clinical characteristics of cervical GTN and to explore diagnostic and therapeutic strategies.
Thirteen patients diagnosed with primary cervical GTN at the Department of Gynecology, Peking Union Medical College Hospital, Beijing, China, between June 1, 1988 and May 31, 2020 were included in the study. All patients had irregular vaginal bleeding, including six who presented with massive bleeding. Seven patients (53.8%) were initially misdiagnosed with a cervical pregnancy. All patients received chemotherapy; 11 (84.6%) also underwent hysterectomy because of chemoresistant lesions or uncontrolled bleeding. All patients achieved complete remission; however, two women (15.4%) experienced a relapse during the median follow-up period of 35 months. A comprehensive review of English-language literature published between 1980 and 2020 identified 22 case reports encompassing 27 patients. The definitive diagnosis was achieved via pathology in 26 of them (96.3%), and hysterectomy was performed in 21 (77.8%).
Owing to its rarity and nonspecific symptoms, the diagnosis of primary cervical GTN is challenging and often relies on pathology. The combination of chemotherapy and hysterectomy is the main therapeutic strategy for this disease.
原发性宫颈妊娠滋养细胞肿瘤（GTN）是极为罕见的异位 GTN。由于其罕见性，此类病变临床诊断困难，以非特异性原因引起的异常阴道出血为最常见症状。为此，本回顾性研究旨在确定宫颈 GTN 的临床特征，并探讨其诊断和治疗策略。
本研究纳入了 1988 年 6 月 1 日至 2020 年 5 月 31 日期间在北京协和医院妇科诊断为原发性宫颈 GTN 的 13 例患者。所有患者均有不规则阴道出血，包括 6 例大出血患者。7 例（53.8%）患者最初被误诊为宫颈妊娠。所有患者均接受化疗；11 例（84.6%）因耐药病灶或无法控制的出血而接受了子宫切除术。所有患者均达到完全缓解；然而，2 名女性（15.4%）在中位 35 个月的随访期间复发。对 1980 年至 2020 年间发表的英文文献进行全面回顾，共纳入 22 例病例报告，涵盖 27 例患者。其中 26 例（96.3%）通过病理明确诊断，21 例（77.8%）患者行子宫切除术。
由于其罕见性和非特异性症状，原发性宫颈 GTN 的诊断具有挑战性，通常依赖于病理学。化疗联合子宫切除术是该疾病的主要治疗策略。
