Laparoscopic management of vaginal clear cell adenocarcinoma arising in pelvic endometriosis: Case report and literature review Ali Mahdavi, MD, Alireza A. Shamshirsaz, MD, Michele Peiretti, MD, Konstantin Zakashansky, MD, Muhammad T. Idrees, MD, and Farr Nezhat, MD From the Division of Gynecologic Oncology and Minimally Invasive Surgery, Department of Obstetrics, Gynecology and Reproductive Science (Drs. Mahdavi, Shamshirsaz, Peiretti, Zakashansky, and Nezhat); and the Department of Surgical Pathology (Dr. Idrees), Mount Sinai School of Medicine, New York, New York. Abstract: Vaginal clear cell adenocarcinoma arising from pelvic endometriosis has not been reported in the literature. We report a case of a 50-year-old woman with stage I clear cell adenocarcinoma of the vagina who was found to have endometriosis adjacent to the vaginal tumor. She was treated with neoadjuvant chemoradiation, laparoscopically assisted radical vaginal hysterectomy, radical upper vaginectomy, and pelvic lymphadenectomy followed by combination chemotherapy. © 2006 AAGL. All rights reserved. KEYWORDS: Vaginal clear cell adenocarcinoma; Endometriosis; Laparoscopic-assisted radical vaginal hysterectomy The incidence of primary vaginal carcinoma is very low; only 1% to 2% of all malignancies of the female genital organs originate from the vagina. Clear cell adenocarci- noma (CCA) of the vagina is a rare vaginal disease, ac- counting for 5% to 10% of primary vaginal malignancies. Although the vast majority of vaginal CCAs have been related to maternal intrauterine exposure to diethylstilbes- trol (DES) 1 and have occurred in young women between the ages of 15 and 27 years, women without history of prenatal DES exposure and those over age 40 have also been af- fected by this neoplasia. Cases of CCA of the rectovaginal septum arising from pelvic endometriosis have been de- scribed in the medical literature. 2 However, the association of vaginal CCA and pelvic endometriosis is less clear. We report on a 50-year-old woman with stage I vaginal CCA and pelvic endometriosis adjacent to the tumor who underwent laparoscopic-assisted radical vaginal hysterec- tomy (LARVH), radical upper vaginectomy, and bilateral pelvic lymphadenectomy after neoadjuvant chemoradiation therapy. To the best of our knowledge, this is the first reported case of vaginal CCA adjacent to an endometriotic lesion, and also the first reported case of this particular tumor to be treated laparoscopically. Case report A 50-year-old nulligravida woman had postmenopausal bleeding for 2 months. She had been menopausal for 2 years with no history of hormone replacement therapy. Gyneco- logic history was positive for severe pelvic endometriosis, diagnosed and treated laparoscopically at age 35, and oral contraceptive use for 15 years. She had a history of rheu- matoid arthritis, psoriasis, and viral hepatitis A, and she had Corresponding author: Farr Nezhat, M.D., Division of Gynecologic Oncology, Mount Sinai Medical Center, 1176 Fifth Avenue, Box 1173, New York, NY 10036. E-mail: farr.nezhat@mssm.edu Submitted September 26, 2005. Accepted for publication January 17, 2006. 1553-4650/$ -see front matter © 2006 AAGL. All rights reserved. doi:10.1016/j.jmig.2006.01.011 Journal of Minimally Invasive Gynecology (2006) 13, 237–241