Symptomatic Round Ligament Leiomyoma in a Patient with Müllerian Agenesis

Abstract

Subject

MRKH syndrome is characterized by the congenital absence of the uterus, cervix, and upper two-thirds of the vagina. This anomaly affects 1 in 5000 females and is thought to arise in-utero between the sixth and twelfth week of gestation when the paramesonephric ducts fail to develop.¹ The round ligament originates from the embryonic gubernaculum and is, therefore, intact in women with MRKH syndrome. Fibroids of the round ligament are very rare. They more commonly originate from the extraperitoneal portion of the round ligament (inguinal canal or labia majora) but can be intraperitoneal in ∼ one-third of patients.² This article presents a case of a symptomatic, intraperitoneal, pedunculated, round ligament fibroid in a patient with MRKH syndrome. To the current authors' knowledge, there are only 5 such patients previously reported in the literature.^1–5

Case

A 40 year-old patient known to have MRKH syndrome presented to the current authors' clinic with left lower-quadrant discomfort and pelvic pressure of 1 year's duration. She had been diagnosed with Müllerian agenesis at age 21 and undergone successful vaginal reconstruction in 1999 in this institution. At that time, she was investigated thoroughly, including an unremarkable pelvic ultrasound. She was lost to follow-up until this new presentation when a large, nontender, mobile solid mass was felt in her left lower quadrant on bimanual pelvic examination. Transvaginal USG detected a 7 × 5–cm, heterogeneous, well-demarcated solid mass in her left lower quadrant, reaching the midline. The mass appeared to be separate but abutting the adjacent left ovary. No abnormality was noted in the right adnexa. This patient's serum cancer antigen (CA)–125 level was normal (6.9 U/mL). MRI was performed, revealing similar findings suggestive of a left leiomyoma, measuring 6 cm in its largest diameter (Fig. 1). She was counseled and agreed to proceed with laparoscopic myomectomy and risk-reducing bilateral salpingectomy (BS).

FIG. 1.

Magnetic resonance image of the leiomyoma (**) originating from the left round ligament (*) in a patient with Müllerian agenesis.

Intraoperatively, a pedunculated, solid round mass was noted arising from the left round-ligament stump. The mass was clearly distinct from the adjacent left ovary and fallopian tube (Fig. 2). Using a bipolar sealing device, the stalk of the myoma was desiccated and subsequently transected with endo-shear scissors. Using monopolar energy, the round ligament stump was then transected to prevent any future recurrences of the same mass. Risk-reducing BS was then performed. To prevent potential complications of mechanical morcellation, the specimen was morcellated into a bag, and the Alexis Contained Extraction System^® (Applied Medical) was used. The surgery was completed without any complications and with minimal blood loss. The official pathology report confirmed that this patient had a leiomyoma (Fig. 3) and benign fallopian tubes. The patient tolerated this procedure well and was discharged to go home in a stable condition on postoperative day 1.

FIG. 2.

Laparoscopy showing the leiomyoma (**) originating from the left round ligament (*) with a clearly separate left ovary (arrow) in a patient with Müllerian agenesis.

FIG. 3.

Histologic section (with hematoxylin and eosin staining) showing typical benign spindle-shaped myocytes with cigar-shaped nuclei (arrow) in a patient with Müllerian agenesis.

Review

A thorough literature review was conducted and 40 cases of leiomyomas in women with MRKH syndrome were identified (Table 1).^1–37 Most of these leiomyomas originated from hypoplastic uterine remnants. Only 5 were described as originating from the round ligament stump, making the current patient the sixth to be reported in the literature.^1–5 Patients with this condition can be asymptomatic and diagnosed incidentally at the time of pelvic imaging, while other patients can present with chronic pelvic pain, pressure symptoms, increased abdominal girth, or acute pain due to torsion of the leiomyoma around the ipsilateral adnexa.

Table 1.

Literature Review of Leiomyomas in Patients with Müllerian Agenesis

1st author, yr & reference	Presenting symptoms	Preoperative diagnosis	Management	Origin of fibroid	Size (cm)
Amaratunga 2017,¹	Chronic pain	Pelvic mass of uncertain origin	Laparotomy	Right round ligament	4.5 × 4
Salem Webbe, 2016²	Incidental	Pelvic mass	Laparotomy	Round ligament	7 × 9
Rhee, 1999³	Enlarging mass	Hard mass with MRKH syndrome	Laparoscopy	Round ligament	10 × 10 × 6
Tsin, 2000⁴	Pain	Solid, midline pelvic tumor	Laparoscopy	Broad, round, & utero-ovarian ligaments	8
McIntyre, 2001⁵	Pelvic mass	Fibroid	Laparotomy	Left adnexa & round ligament	15
Powell B, 1988⁶	Pain	Hematometra	Laparotomy	Left uterine horn	4
Baker, 1995⁷	Pelvic mass	Fibroid uterus	Laparotomy	Uterine remnant	16.5 × 14 × 10.5
Hsu, 1999⁸	Gradual abdominal distention	Ovarian cancer	Laparotomy	Left uterine remnant	29 × 25 × 8.5
Dandu, 2000⁹	Chronic pain	Hematometra	Laparoscopy; then laparotomy	Rudimentary uterine horn	3 × 6
Galajdova, 2003¹⁰	Acute pain	Large pelvic tumor	Laparotomy for torsion in 1997	Right rudimentary horn	8 × 6 × 5
Galajdova, 2003¹⁰	Growing pelvic mass	Growing fibroid	Laparotomy for recurrence in 2002	Right rudimentary horn	10 × 7.5
Edmonds, 2003¹¹	Increasing abdominal swelling & urinary frequency	Fibroid	Laparotomy	Right rudimentary horn	10
Jadoul, 2004¹²	Dyspareunia	Fibroid	Laparoscopy	Left rudimentary uterine horn	9.8 × 7.6 × 8.0
Deligeoroglou, 2004¹³	Chronic pain	Solid mass	Laparoscopy	Left uterine remnant	5.9 × 5.5
Deligeoroglou, 2004¹³	Incidental	Left adnexal mass	Laparoscopy	Left uterine remnant	4.8 × 3.6
Goldstein, 2004.¹⁴	Shortness of breath, increased abdominal girth, early satiety	Solid pelvic mass	Laparotomy	Rudimentary uterus	15.0 × 10.0 × 10.0
Roy, 2005¹⁵	Pelvic mass	MRKH syndrome with 2 pelvic masses	Laparotomy	Right & left rudimentary uterine horns	12 × 10 & 5 × 5
Petrić, 2008¹⁶	Pain & enlarging mass	Pelvic tumor torsion	Laparotomy	Could not be identified	Not reported
Papa, 2008¹⁷	Pain & pelvic swelling	Pelvic mass	Laparotomy	Left uterine remnant	15 × 12 × 9.5
Lanowska, 2009¹⁸	Incidental	Pelvic mass	Laparoscopy	Right uterine remnant	8 × 10
Lamarca, 2009¹⁹	Incidental	Multiple fibroids	Laparotomy	Right & left uterine remnant	6 × 3 on the right; 3 on the left
Deka, 2011²⁰	Chronic pain	Right pelvic mass	Laparotomy	Right uterine horn	2.6 × 3
Fletcher, 2012²¹	Incidental; developed acute pain (torsion) 1 day preoperatively	Uterine fibroid	Laparotomy	Müllerian remnant	10 × 15
Fletcher, 2012²¹	Pelvic mass	Fibroid	Laparotomy	Müllerian remnant	6
Rawat, 2013²²	Pain & pelvic mass	Large fibroid with MRKH syndrome	Laparotomy	Rudimentary uterus	25 × 18 × 12
Salman, 2913²³	Incidental	Fibroidlike mass	Laparoscopy	Left rudimentary uterine horn	6 x 5
Valecha, 2014²⁴	Chronic pain & pelvic mass	Multiple fibroids	Laparotomy	Left rudimentary uterine horn	6 × 6
Guhan, 2014²⁵	Acute pain	Fibroid	Laparotomy + bilateral rudimentary horns removal	Right rudimentary uterine horn	10 × 10
Kundu, 2014²⁶	Acute pain	Torsion	Laparotomy	Rudimentary uterine horns bilaterally	10 on the right & 9 on the left
Girma, 2015²⁷	Chronic pain & lower-abdominal swelling	Ovarian tumor	Laparotomy	Right uterine horn remnant	18 × 10 × 8
Kulkarni, 2015²⁸	Incidental	MRKH syndrome associated with right adnexal mass	Laparotomy- hysterectomy	Rudimentary uterine horn	5 × 4 × 4
Vidyashree, 2015²⁹	Pain	MRKH syndrome with multiple fibroids, &torsion on the right	Laparotomy	Müllerian remnant	6 × 7 & 5 × 6
Hasegawa, 2015³⁰	Growing abdominal mass	Fibroids or GI stromal tumor	Laparotomy	Rudimentary uterine horns bilaterally	20 × 30
Narayanan, 2015³¹	Pain	MRKH syndrome with multiple fibroids, with areas of necrosis arising from the Müllerian remnants & torsion of right ovary	Laparotomy of bilateral masses	Müllerian remnant uterine tissue	Not reported
Park, 2016³²	Pelvic mass	Fibroids	Laparotomy	Rudimentary uterus & left fallopian tube	16 × 9.2 × 10
Dimitriadis, 2016³³	Incidental	Fibroids	Laparotomy	Left uterine remnant	7.2
Hoo, 2016³⁴	Abdominal mass & pain	Fibroid or ovarian tumor	Laparotomy	Right broad ligament	15 × 13 × 13
Yi, 2017³⁵	Acute pain and pelvic masses.	Solid tumor, along with cystic tumor	Laparoscopy + BSO	Uterine remnant	8.1 × 7.9 × 7.2
Sharma, 2017³⁶	Chronic pain & pelvic mass	Bilateral ovarian neoplasms	Laparoscopy	Right & left rudimentary horns	18 × 15 & 5 × 4
Deepika, 2017³⁷	Pelvic mass	Multiple fibroids with Müllerian anomaly	Laparotomy–hysterectomy	Right-sided horn fibroid	15 × 8 × 8

yr, year; MRKH, Mayer–Rokitansky–Küster–Hauser; BSO, bilateral salpingo-oophorectomy; GI, gastrointestinal.

Trainee's Perspective

Women with Müllerian agenesis lack uteri. Therefore, when these women present with solid pelvic masses, trainees are unlikely to consider leiomyomata in the differential diagnosis, as those are most-often uterine in origin. The trainees will first think of an adnexal mass, irrespective of the patient's clinical presentation, leading to unnecessary testing, laparotomy, and heightened patient anxiety. This can be avoided with the knowledge that women with Müllerian agenesis might have rudimentary uterine horns containing nonfunctioning myometrial tissue and, rarely, active endometrium that, therefore, could develop leiomyomas and sometimes even adenomyosis and endometriosis.^34,38 Moreover, women with Müllerian agenesis have an intact round ligament that could also be the origin of leiomyomata.

Faculty's Perspective

Of the 40 published cases on leiomyomas in women with MRKH syndrome, several were misdiagnosed preoperatively as being adnexal tumors.^{1,4,8,10,16,17,27,30,34,35} For the current patient, the current authors did first think of a possible adnexal mass and thus requested a CA-125 level test. However, the normal-looking ovary adjacent to the pelvic mass on USG was highly suggestive of a leiomyoma. This was subsequently confirmed by pelvic MRI.

Whether the leiomyoma originates from the uterine remnant or round-ligament stump, the mass is almost often pedunculated in nature. The most-common presentation cited in the literature is that of pelvic pain. Acute torsion of the adnexa around the leiomyoma pedicle has been described in 7 patients, necessitating emergent surgery to salvage the affected ovary.^{10,16,26,29,31,34,35} Myxoid degeneration of a large leiomyoma has also been described in a patient with Müllerian agenesis.³⁶

When it comes to management, the current authors do recommend removal of the leiomyoma—especially if it is symptomatic. Considering the possibility of torsion, the current authors also advise removing large asymptomatic leiomyomata—especially in younger women in need of ovarian function. Most cases reported in the literature were managed by laparotomy. The current authors chose a laparoscopic approach to benefit the patient with the well-established advantages of minimally invasive surgery, including quick recovery, decreased postoperative pain, decreased hospital stay, and lower incidence of postoperative complications. Enclosed morcellation in a bag was performed, as this has become the standard of care ever since the U.S. Food and Drug Administration issued recommendations on the cautious use of power morcellation.³⁹ In fact, leiomyosarcomas, although rare, could arise from the round ligament and, therefore, extrauterine fibroids should not be presumed to be benign.⁴⁰ Following resection of the fibroid, we resected the round ligament stumps to prevent potential recurrence of the myoma in the future. Recurrence of uterine remnant fibroids has been reported, necessitating reoperation.¹⁰

In conclusion, it is important for trainees to know that a solid pelvic mass in a woman with Müllerian agenesis is not always ovarian in origin. The mass could be a leiomyoma originating from the round ligament or from Müllerian remnants. Diagnosis can be made by pelvic USG and MRI, which are helpful for distinguishing a leiomyoma from any ovarian pathology. Laparoscopic resection is feasible and recommended to avoid potential complications, such as torsion.

Footnotes

Authors' Contributions

Dr. Hamze is the trainee who was primarily involved in the care of this patient. He drafted the article, together with Drs. Mourad and Chamsy, and conducted a thorough literature review. Dr. Mourad, another trainee, also cared for this patient. Dr. Seoud was the primary faculty member who initially evaluated the patient in a clinic and conducted the work-up. He also obtained the patient's consent to be included in this article. He contributed to the conception of the work, together with Dr. Chamsy, and reviewed the article. Dr. Chamsy operated on the patient and was involved in data acquisition. All of the authors gave final approval of the article.

Author Disclosure Statement

No financial conflicts of interest exist.

Funding Information

No funding was received for this article.

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