Uterine leiomyomas are a common finding in medical practice, but their frequency changes drastically when contextualized in a syndrome, as in the following case A 50-year-old woman with a known Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome presented at our clinic with abdominal pain located in the lower quadrants and scarcely responsive to analgesic therapy A twisted gynecological pelvic mass was diagnosed, and management for prompt resolution was adopted Histologically the mass was described as a leiomyoma The aim of the present study is to share our experience and to review the literature to compare different manifestation of the disease and different approach used in the various centers The additional novelty of the paper is the immunohistochemical study we carried out on the leiomyoma that is contrasted with the current etiopathogenetic theories

The Rare, Unexpected Condition of a Twisted Leiomyoma in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Etiopathogenesis, Diagnosis and Management. Our Experience and Narrative Review of the Literature

A rare case of multiple leiomyomas on rudimentary uterus in a woman with Mayer Rokitansky Kuster Hauser (MRKH) syndrome: A challenging diagnosis and laparoscopic approach.

BACKGROUND Patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome are infertile secondary to hypoplasia or complete agenesis of the uterus, yet they remain at risk of primary neoplasms of the ovaries because embryologically the uterus and ovaries develop via separate mechanisms. CASE A 72-year-old nulliparous woman with a history of primary amenorrhea underwent an exploratory laparotomy for a suspected uterine fibroid. In addition to the pelvic mass, the patient was found to have findings consistent with MRKH syndrome. Postoperative pathological examination demonstrated bilateral ovarian Sertoli cell tumors. CONCLUSIONS The case presented is unique in that 2 rare pathologies, bilateral Sertoli cell tumors of the ovary and MRKH syndrome, developed concomitantly in the same patient.

Mayer-Rokitansky-Küster-Hauser Syndrome With Bilateral Ovarian Sertoli Cell Tumors: Review of the Literature and Report of a Rare Case.

Round ligament tumours represent a rare entity that can present similarly to an incarcerated hernia. Basic understanding and appropriate preoperative management is imperative in order to differentiate between the two diagnoses. Leiomyoma is the most common type of round ligament tumour. It is associated with oestrogen exposure and is more common in the presence of uterine leiomyomas. Here we discuss a 68-year-old woman who presented with a palpable left inguinal mass that progressively grew in size, associated with pelvic pressure and discomfort. On surgical resection, the mass was found to be derived from the round ligament at the entrance of the external inguinal ring. Pathology confirmed a round ligament leiomyoma, measuring 25×9×8.5 cm. This case is the largest round ligament leiomyoma recorded to date and the first to exhibit carneous degeneration. A review of the current literature is also provided.

https://casereports.bmj.com/content/casereports/2017/bcr-2017-222454.full.pdf

Large, extra-abdominal leiomyoma of the round ligament with carneous degeneration

I n September 2018, a 48-year-old woman with a 2-month history of inflammatory low back pain was referred to our center. Her family history for rheumatic diseases was unremarkable. She also complained of amenorrhea in the past 11 weeks, which had been thought to be perimenopause-related. Clinical examination ruled out pregnancy, whereas sacroiliac joints provocative tests were positive. Etoricoxib 90 mg once daily was started with minor improvement after 3 weeks. Acute phase reactants as well as sacroiliac joints radiographs were found to be normal. HLA-B27 testing was negative also. Magnetic resonance imaging (MRI) of sacroiliac joints showed subchondral bone marrow edema of the right sacral wing. Nevertheless, enlarged uterus with an intrauterine mass with cystic space without associated fetus was also reported. The patient was referred to the Obstretrics and Gynecology Division. Her serum human chorionic gonadotropin level was greater than 200,000 mUI/mL. Ultrasound-guided suction and curettage were carried out, and consequent histopathological analysis indicated complete hydatidiform mole. Serum human chorionic gonadotropin level was assessed every 1 week until normalization, which occurred 6 weeks later. Consistently, low back pain resolved the week after surgery and never flared again. She did not undergo follow-up MRI because she was asymptomatic.

Complete Hydatidiform Mole Mimicking Sacroiliitis.

Background The occurrence of an extra-uterine leiomyoma, arising from the intra-peritoneal portion of the round ligament in a lady with Mullerian agenesis diagnosed at the age of forty is extremely rare. We report a case of this rare combination in a Middle Eastern woman. Case A 40 years old lady, primarily amenorrheic, presented to our clinic for an infertility consultation. The work- up showed features suggestive of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with a leiomyoma arising from the intra-peritoneal part of the round ligament.

Intra-peritoneal leiomyoma of the round ligament in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

The management of the obstructed hemivagina and ipsilateral renal anomaly, or OHVIRA syndrome, requires vaginal access compromising sometimes the hymenal integrity. A vaginoplasty is the m...

A Novel Surgical Intervention for an Uncommon Entity: Laparoscopy-Assisted Resection of a Vaginal Septum in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome.

Michel Samaha

Papers

Intra-peritoneal leiomyoma of the round ligament in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

A Novel Surgical Intervention for an Uncommon Entity: Laparoscopy-Assisted Resection of a Vaginal Septum in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome.

Uterine choriocarcinoma diagnosed 11 years after menopause: A case report.