Fibroid originated from rudimentary horn in mullerian agenesis
Keywords:Agenesis, Histopathology, Mulleriananamoly, Post-operative period
Mullerian agenesis or Mayer Rokitansky- Kuster- Hauser syndrome is defined by congenital absence of both the uterus and vagina. It affects 1 in 4000-5000 females. Leiomyoma arising from the uterine remanent in case of MRKH is very rare. Very few cases have been reported in literature. The exact etiopathogenesis of leiomyoma from the rudimentary uterus in MRKH syndrome is not known. We are reporting a rare case of 35-year-old married, nulliparous female who presented with primary amenorrhea and lump in lower abdomen since, 1 year. On physical examination, a mass of 18-week size of uterus, firm in consistency, irregular, nontender, mobile from side to side, occupying right iliac and hypogastric region was detected. Provisional diagnosis of multiple leiomyoma with mullerian anomaly was made on USG and MRI for which laparotomy was performed. Per-operatively, there was a mass of approx. 15 x 8 x 8 cm consisting of multiple fibroids present, uterus not visualized separately. Right ovary and tube absent. Left fallopian tube and ovary were normal arising from left rudimentary horn with no endometrial tissue. Blind end with no cervix. Partial agenesis of vagina. No communication possible between rudimentary horn and vagina. Patient underwent hysterectomy in view of non-functioning uterus. Histopathology report confirmed the diagnosis. Finally, it was diagnosed as a case of multiple leiomyoma originating from uterine remanent. Post-operative period was uneventful.
Welt CK, Barbieri RL. Etiology, diagnosis, and treatment of primary amenorrhea. Upto Date [updated Apr 14, 2009].[Links]. 2007.
Rawat KS, Buxi TBS, Yadav A, Ghuman SS, Large SD. Leiomyoma in a woman with MRKH syndrome. J Radiol Case Rep. 2013;7(3):39-46.
Nirmala Duhan, Yogender S Kadian, Urmila Duhan, Nidhi Rajotia. Mullerian, Renal and Skeletal Anomalies A Rare Association. JK Sci. 2009;11(2).
Willimsen WN. Combination of the Mayer-Rokitansky-kuster and Klippel Fiel Syndrome-a case reportand literature review.Eur J Obstet Gynecol Rerod Biol. 1982;13:229-35.
Lico D, Morelli M, Venturella R, Mocciaro R, Di Cello A, Rania E, Zullo F. An Unusual Extremely Distant Non-Communicating Uterine Horn with Myoma and Adenomyosis Treated with Laparoscopic Hemy-Hysterectomy. J Minimally Invasive Gynecol. 2013;20(6):S187.
Kallol K Roy, Suman Lal, Neelam Banerjee Large leiomyomas in Mayer-Rokitansky-KüsterHauser syndrome. J Obstet Gynecol India. 2005;55(2):183-4.
Singh S, Chakravarty B, Chakravarty M, Chakravarty A. Large Fibroid arising from Mullerian Remnant Mimicking as Ovarian Neoplasm in a Woman with MRKH Syndrome. Int J Infert Fetal Medic. 2012;3(1):30-2.
Lanowska M, Favero G, Schneider A, Kohler C Laparoscopy for differential diagnosis of a pelvic mass in a patient with Mayer-Rokitanski-Küster-Hauser (MRKH) syndrome. Fertil Steril. 2009;91(3):931.e17-8.