Lei Xian-Gao, Zhang Heng
Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education; West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
World J Clin Oncol. 2024 Oct 24;15(10):1359-1365. doi: 10.5306/wjco.v15.i10.1359.
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare Müllerian duct anomaly, characterized by a combination of urogenital abnormalities. The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is exceptionally rare, posing significant challenges for screening, early diagnosis, and effective management.
We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome. The patient presented with irregular vaginal bleeding for 4 years. On gynecological examination, an oblique vaginal septum was suspected. Surgical resection of the vaginal septum revealed a communicating fistula and a tumor on the left vagina and the left side of the septum, which was confirmed as clear cell carcinoma. One month later, she underwent a radical hysterectomy, vaginectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Due to significant side effects, she completed only one course of chemotherapy. A year later, lung metastasis was detected and continued to grow. A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery. We also conducted a systemic review of the literature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.
Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult, and require early surgical intervention and regular imaging surveillance.
赫林-韦纳-温德利希（HWW）综合征是一种罕见的苗勒管异常，其特征为泌尿生殖系统异常的组合。HWW综合征患者发生原发性宫颈阴道癌极为罕见，这给筛查、早期诊断和有效管理带来了重大挑战。
我们报告了一例罕见的40岁患有HWW综合征的女性并发原发性阴道透明细胞癌的病例。患者出现不规则阴道出血4年。妇科检查怀疑有斜行阴道纵隔。手术切除阴道纵隔后发现一个交通瘘以及左侧阴道和纵隔左侧有一个肿瘤，经确诊为透明细胞癌。一个月后，她接受了根治性子宫切除术、阴道切除术、双侧输卵管卵巢切除术和盆腔淋巴结清扫术。由于严重的副作用，她仅完成了一个疗程的化疗。一年后，检测到肺转移且持续进展。初次手术后4年进行了胸腔镜右上叶楔形切除术。我们还对HWW综合征中原发性宫颈癌或阴道癌的文献进行了系统综述，以探究这一罕见病症。
HWW综合征患者的宫颈阴道腺癌较为隐匿，需要早期手术干预和定期影像学监测。
