Primary invasive vaginal cancer in the setting of the Mayer-Rokitansky-Kuster-Hauser syndrome.
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.


METHODS
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.


CONCLUSIONS
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.
Tewari
Devansu S
DS
Division of Gynecologic Oncology, Division of Radiation Oncology, Chao Family Comprehensive Cancer Center, University of California Irvine Medical Center, 101 The City Drive, Orange, CA 92868, USA.
McHale
Michael T
MT
Kuo
Jeffrey V
JV
Monk
Bradley J
BJ
Burger
Robert A
RA
D000015:Abnormalities, Multiple / Q000473:pathology*; D000328:Adult; D018269:Carcinoma, Endometrioid / Q000150:complications / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D013577:Syndrome; D014599:Uterus / Q000002:abnormalities; D014621:Vagina / Q000002:abnormalities; D014625:Vaginal Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy