CASE REPORT Leiomyoma Arising from Mullerian Remnant, Mimicking Ovarian Tumor in a Woman with MRKH Syndrome and Unilateral Renal Agenesis

Girma, Wubishet; Woldeyes, Wondimagegnehu Sisay

doi:10.4314/ejhs.v25i4.14

Cited by 16 publications

(19 citation statements)

References 6 publications

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“…[1][2][3][4][5][6][11][12][13] Differential diagnosis of leiomyoma of rudimentary uterus in MRKH syndrome includes ovarian fibroma, gastrointestinal stromal tumor (GIST) of intestine and extravesical leiomyoma of urinary bladder. The uncommon occurrence of leiomyoma in MRKH may be explained by decreased concentration or sensitivity of estrogen receptors in the rudimentary tissue.…”

Section: Discussionmentioning

confidence: 99%

“…The prevalence of congenital uterine anomalies appears to be 6.7% in fertile population, and it is found in 7.3% of an infertile population. 1 Occurrence of a myoma arising from a mullerian remnant is an extremely rare finding and only few cases reported in literatures. [1][2][3][4][5][6] The incidence of associated urologic abnormalities ranges between 15-40%, and skeletal anomalies such as congenital fusion or absence of vertebra occur in approximately 12-50% of cases.…”

Section: Introductionmentioning

confidence: 99%

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Fibroid with unilateral streak ovary in Mullerian Agenesis!a rare case report

Malik¹,

Goyal

Bhardawaj

2018

Int J Reprod Contracept Obstet Gynecol

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Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare anomaly (1/5000) associated with uterine and vaginal aplasia with normal ovaries. A fibroid in a normal uterus is common but a fibroid arising from rudimentary horn in Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is very rare. It is also rare to have the association of unilateral ovarian dysgenesis in MRKH. A 37 year old married female with primary amenorrhea presented to the OPD of Dr. RML Hospital with abdominopelvic mass for one year. She was phenotypically normal looking and there was no other associated cardiac, ocular or skeletal abnormality. A large 15 × 15 cm abdominopelvic mass was present in suprapubic area which was firm in consistency and non-tender. A Two and a half inch long blind vaginal pouch was present with absence of cervix and uterus. A provisional diagnosis of solid ovarian tumor in MRKH syndrome was made. Her investigations -Serum FSH, LH, estradiol and tumor markers were normal. Her ultrasound and MRI reported a fibroid with normal ovaries. Laparotomy revealed absence of uterus and cervix and a large fibroid arising from right rudimentary horn with left streak ovary. Right ovary was enlarged to 4 × 4 cm, showing multiple small follicles. Fibroid along with the rudimentary horns and the streak ovary was removed, preserving the normal right ovary. Histopathology confirmed the diagnosis of leiomyoma in rudimentary horn with left streak ovary, making it class1 type b anomaly under American fertility society classification. Preoperative assessment of rare anomalies can be different from intraoperative findings in certain female reproductive tract anomalies. Awareness of rare combinations can help in judicious management of such cases.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fibroid with unilateral streak ovary in Mullerian Agenesis!a rare case report

Malik¹,

Goyal

Bhardawaj

2018

Int J Reprod Contracept Obstet Gynecol

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“…In the most recent publications on MRKH syndrome, we could find cases reported on leiomyoma, adenomyosis, but ovarian tumor is rare in MRKH syndrome and difficult to diagnose if it is not bulky. [11][12][13][14] The incidence of the combination of MRKH with ovarian tumors was not reported. However, a review of the literature demonstrated nearly a dozen reported cases of MRKH syndrome associated with ovarian tumors (Table 1).…”

Section: Diagnosismentioning

confidence: 99%

Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Adjoby¹,

Koffi²,

Effoh³

et al. 2020

Int J Reprod Contracept Obstet Gynecol

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Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is an unknown congenital etiology disorder characterized by agenesia or hypoplasia of the Müller ductal system, including the upper vagina, uterus and fallopian tubes. The occurrence of an associated ovarian tumor is rare, with fewer than 20 cases reported to date according to the literature. We report the case of a 14-year-old girl, virgin, who had not yet seen her menarche, complaining of an abdomino-pelvic mass associated with pain. The ultrasound performed revealed a large left ovarian tumor and an absence of uterus. The indication of a laparotomy confirmed the ovarian mass and a complete absence of uterus associated with vaginal hypoplasia. The contralateral ovary was present, and of normal appearance. The pathological examination was in favor of a mature benign multi-tissular teratoma. This is the first case described in our service. The mode of transmission of this entity appears to be autosomal dominant with low penetrance and variable expressivity, suggesting that the incidence of this syndrome is likely underestimated. With the development of techniques of medical assistance to procreation, maternity remains possible, particularly through gestational surrogacy.

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“…These patients are characterized by a normal female karyotype, secondary sexual characteristics, and ovarian function [2]. As ovarian function is normal in most patients with MRKH syndrome, the development of estrogen-dependent pathologies, such as leiomyomas and adenomyosis, is possible in the rudimentary uterine horn [3,4] or in other pelvic areas [5]. Here, we report a case with severe abdominal pain that was diagnosed as torsion of a right rudimentary uterine horn with multiple leiomyomas in a patient with MRKH syndrome.…”

Section: Introductionmentioning

confidence: 99%

Torsion of a rudimentary uterine horn with multiple leiomyomas in a case of Mayer-Rokitansky-Küster-Hauser syndrome

Kim¹,

Na²,

Kim³

et al. 2020

Clin. Exp. Obstet. Gynecol.

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The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital hypoplasia or aplasia of the uterus and vaginal agenesis. A 53-year-old woman presented to the emergency department with severe abdominal pain. She was diagnosed with torsion of the right rudimentary uterine horn with multiple leiomyomas with MRKH syndrome. The twisted rudimentary uterine horn and multiple leiomyomas were successfully removed via laparoscopy. MRKH syndrome with uterine horn torsion is uncommon and ours is the first case of torsion in MRKH syndrome treated via a laparoscopic approach. Our report indicates that laparoscopy is ideal for the simultaneous diagnosis and treatment of pelvic masses in MRKH syndrome.

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CASE REPORT Leiomyoma Arising from Mullerian Remnant, Mimicking Ovarian Tumor in a Woman with MRKH Syndrome and Unilateral Renal Agenesis

Cited by 16 publications

References 6 publications

Fibroid with unilateral streak ovary in Mullerian Agenesis!a rare case report

Fibroid with unilateral streak ovary in Mullerian Agenesis!a rare case report

Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Torsion of a rudimentary uterine horn with multiple leiomyomas in a case of Mayer-Rokitansky-Küster-Hauser syndrome

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