Rare Case of Leiomyoma and Adenomyosis in Mayer-Rokitansky-Kuster-Hauser Syndrome

Hoo, Pek Sung; Norhaslinda, A. R.; Reza, J. N. Shah

doi:10.1155/2016/3725043

Cited by 18 publications

(22 citation statements)

References 4 publications

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“…For example, adenomyosis lesions were reported in myometrium of Rokitansky-Kuster-Hauser syndrome patient, who lacked functional endometrium. 55,56 Alternative hypotheses propose that ectopic endometrium derives by metaplasia de novo of embryonic epithelial progenitors (remnants) or differentiation of adult endometrial stem cells that transit to the myometrium.…”

Section: De Novo Metaplasia Theorymentioning

confidence: 99%

Adenomyosis: Mechanisms and Pathogenesis

et al. 2020

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Adenomyosis is a common disorder of the uterus, and is associated with an enlarged uterus, heavy menstrual bleeding (HMB), pelvic pain, and infertility. It is characterized by endometrial epithelial cells and stromal fibroblasts abnormally found in the myometrium where they elicit hyperplasia and hypertrophy of surrounding smooth muscle cells. While both the mechanistic processes and the pathogenesis of adenomyosis are uncertain, several theories have been put forward addressing how this disease develops. These include intrinsic or induced (1) microtrauma of the endometrial–myometrial interface; (2) enhanced invasion of endometrium into myometrium; (3) metaplasia of stem cells in myometrium; (4) infiltration of endometrial cells in retrograde menstrual effluent into the uterine wall from the serosal side; (5) induction of adenomyotic lesions by aberrant local steroid and pituitary hormones; and (6) abnormal uterine development in response to genetic and epigenetic modifications. Dysmenorrhea, HMB, and infertility are likely results of inflammation, neurogenesis, angiogenesis, and contractile abnormalities in the endometrial and myometrial components. Elucidating mechanisms underlying the pathogenesis of adenomyosis raise possibilities to develop targeted therapies to ameliorate symptoms beyond the current agents that are largely ineffective. Herein, we address these possible etiologies and data that support underlying mechanisms.

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Section: De Novo Metaplasia Theorymentioning

confidence: 99%

Adenomyosis: Mechanisms and Pathogenesis

et al. 2020

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“…As the ovarian function is normal, the development of estrogen-dependent pathologies is possible in various areas of the pelvis in patients with MRKH syndrome [3][4][5]. A rudimentary uterine horn is a typical site where estrogen-dependent smooth muscle cells are found and is a most likely site for the occurrence of leiomyomas or ade- nomyosis in patients with MRKH syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…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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“…[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%

“…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. Unilateral renal anomalies are associated with 50% of the patients.…”

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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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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Rare Case of Leiomyoma and Adenomyosis in Mayer-Rokitansky-Kuster-Hauser Syndrome

Cited by 18 publications

References 4 publications

Adenomyosis: Mechanisms and Pathogenesis

Adenomyosis: Mechanisms and Pathogenesis

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

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

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