A rare case of MRKH with adenomyosis and literature review.

Objective To investigate the possible pathogenesis,diagnosis and treatment of Mayer-Rokitansky-Kuster-Hauser syndrome(MRKH syndrome) with adenomyosis.Methods We reported a 47-year-old women of MRKH syndrome with adenomyosis treated in Peking Union Medical College Hospital in April 2009,and reviewed the related literature.Results The patient complained of cyclic low abdominal pain for 10 years.Pelvic examination revealed vagina of 6 cm in length,and no cervix and uterus.MRI showed a solid mass in the left pelvic cavity.Serum CA125 was 138.2U/L.Laparoscopy showed adenomyosis of left rudimentary uterine horn with 10 weeks gestational age in size,and right rudimentary uterine horn of 2cm×1.5cm×1cm.Both ovaries and tubes were normal.The left uterine horn was laparoscopically excised.Macroscopic appearance showed adenomyotic change of myometrium without uterine cavity.The histopathological examination confirmed the diagnosis of adenomyosis with positive ER and PR,and positive CD10.Conclusion The development of adenomyosis on uterine remnants is possible in patients with MRKH,may develop from the mullerian remnants.