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.

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.