Unusual manifestation of Mayer–Rokitansky–Küster– Hauser syndrome presenting as bilateral Müllerian remnant myomas with torsion and inguinal prolapse

Myoma in the uterus is a common occurrence, but myoma in Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is extremely rare. We present a case report of a 41-year-old lady with MRKH syndrome who presented to our magnetic resonance imaging (MRI) center with acute abdominal pain for 1 week. MRI revealed a huge, well-circumscribed T2-weighted hypointense solid mass lesion centered in the hypogastrium, with torsion. There was an incidental, similar intensity, smaller lesion in the right inguinal canal. The right ovary was in an ectopic position. A structure suspicious of the left ovary was seen adherent to the torsed pedicle. There was an associated single ectopic pelvic kidney in the pre-sacral region. Structures resembling Müllerian remnants were identified which gave us a clue to the diagnosis of origin of myoma from Müllerian remnants. Imaging diagnosis of a torsed myoma from left Müllerian remnant and an incidental prolapsed myoma in the right inguinal canal originating from right Müllerian remnant was diagnosed. The patient underwent laparotomy and the intra-abdominal tumor was removed. MRI was very valuable to characterize, locate the origin of tumor, and identify the torsion, aiding efficient management.