Primary Invasive Vaginal Cancer in the Setting of the Mayer–Rokitansky–Kuster–Hauser Syndrome

• Published in: Gynecologic oncology Vol. 85; no. 2; pp. 384 - 387
• Main Authors: Tewari, Devansu S., McHale, Michael T., Kuo, Jeffrey V., Monk, Bradley J., Burger, Robert A.
• Format: Journal Article
• Language: English
• Published: San Diego, CA Elsevier Inc 01.05.2002; Elsevier
• Subjects: Abnormalities, Multiple - pathology; Adult; Biological and medical sciences; Carcinoma, Endometrioid - complications; Carcinoma, Endometrioid - pathology; Carcinoma, Endometrioid - therapy; Combined Modality Therapy; Female; Female genital diseases; Gynecology. Andrology. Obstetrics; Humans; Medical sciences; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the genital tract and mammary gland; Syndrome; Tumors; Uterus - abnormalities; Vagina - abnormalities; Vaginal Neoplasms - complications; Vaginal Neoplasms - pathology; Vaginal Neoplasms - therapy; Human; Agenesis; Squamous cell carcinoma; Vagina; Vaginal diseases; Malignant tumor; Female genital diseases; Case study; Pathology; Primitive; Treatment; Uterus; Malformation; Surgery; Rokitansky Kuster Hauser syndrome; Uterine diseases; Adult; Female; Clinical investigation
• ISSN: 0090-8258; 1095-6859
• DOI: 10.1006/gyno.2002.6637

Background. The Mayer–Rokitansky–Kuster–Hauser syndrome occurs in 1 in 4000 to 5000 female births. Primary vaginal cancer constitutes less than 2% of all malignancies of the female genital tract. A report of the first case of the unlikely occurrence of both of these developments in the same patient is presented. Case. A 34-year-old nulligravid Philippine woman with a history of Mayer–Rokitansky–Kuster–Hauser syndrome presented with a 5-month history of bleeding from a blind vaginal pouch. Vaginal biopsy identified a moderately differentiated endometrioid adenocarcinoma. Exploratory laparotomy, bilateral salpingo-oophorectomy, pelvic and iliac lymph node samplings, and excision of a mullerian remnant were performed with no evidence of disease. A FIGO Stage I vaginal cancer was assigned and pelvic irradiation was given. Disease recurred 4 months later and the patient underwent total pelvic exenteration. More than 1 year since the exenteration procedure, she is without evidence of disease. Conclusion. This is the first reported case of a primary vaginal cancer in a patient with Mayer–Rokitansky–Kuster–Hauser syndrome. It is a reminder that routine gynecologic examinations are still warranted as these patients are at risk for malignant changes in residual mullerian tissues.