Lei XG, Zhang H. Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome: A case report. World J Clin Oncol 2024; 15(10): 1359-1365 [PMID: 39473867 DOI: 10.5306/wjco.v15.i10.1359]
Corresponding Author of This Article
Heng Zhang, MD, PhD, Associate Professor, 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, No. 20 South Renmin Road, Chengdu 610041, Sichuan Province, China. mazy6121981@163.com
Research Domain of This Article
Obstetrics & Gynecology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Oncol. Oct 24, 2024; 15(10): 1359-1365 Published online Oct 24, 2024. doi: 10.5306/wjco.v15.i10.1359
Vaginal clear cell adenocarcinoma in Herlyn-Werner-Wunderlich syndrome: A case report
Xian-Gao Lei, Heng Zhang
Xian-Gao Lei, Heng Zhang, 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
Author contributions: Lei XG and Zhang H contributed to manuscript writing and editing, Lei XG and Zhang H contributed to data collection; Lei XG and Zhang H contributed to data analysis; Zhang H contributed to conceptualization and supervision; all authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Heng Zhang, MD, PhD, Associate Professor, 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, No. 20 South Renmin Road, Chengdu 610041, Sichuan Province, China. mazy6121981@163.com
Received: April 26, 2024 Revised: September 14, 2024 Accepted: September 19, 2024 Published online: October 24, 2024 Processing time: 155 Days and 12.2 Hours
Abstract
BACKGROUND
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.
CASE SUMMARY
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.
CONCLUSION
Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult, and require early surgical intervention and regular imaging surveillance.
Core Tip: Cervico-vaginal adenocarcinomas in patients with Herlyn-Werner-Wunderlich (HWW) syndrome are extremely rare. The present case and a literature review of this rare condition, indicate that patients with HWW syndrome may have a higher risk of developing cervico-vaginal adenocarcinomas, compared to the general female population. These cancers typically occur on the obstructed side, making them occult and difficult to detect. Therefore, it is crucial for patients with HWW syndrome to undergo septum resection upon diagnosis or, at a minimum, have regular imaging evaluations of the cervix and vagina, to facilitate early detection and management.
