Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly of the genital tract. Since the secretion of sex hormones from the ovaries is preserved, leiomyomas and adenomyomas, which are estrogen-dependent diseases, may develop from the uterine remnant. In contrast, patients with my...

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Main Authors: Yukihiro Azuma, Koji Yamamoto, Mei Matsumoto, Hiroki Nagata, Ikumi Wada, Keisuke Miyamoto, Fuminori Taniguchi
Format: Article
Language:English
Published: Hindawi Limited 2023-01-01
Series:Case Reports in Obstetrics and Gynecology
Online Access:http://dx.doi.org/10.1155/2023/5182889
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author Yukihiro Azuma
Koji Yamamoto
Mei Matsumoto
Hiroki Nagata
Ikumi Wada
Keisuke Miyamoto
Fuminori Taniguchi
author_facet Yukihiro Azuma
Koji Yamamoto
Mei Matsumoto
Hiroki Nagata
Ikumi Wada
Keisuke Miyamoto
Fuminori Taniguchi
author_sort Yukihiro Azuma
collection DOAJ
description Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly of the genital tract. Since the secretion of sex hormones from the ovaries is preserved, leiomyomas and adenomyomas, which are estrogen-dependent diseases, may develop from the uterine remnant. In contrast, patients with myotonic dystrophy type 1 (DM1), the most common dystrophy in adults, are considered to be at high risk for benign tumors of the female reproductive system, such as uterine leiomyomas and ovarian cysts. A rare case of huge leiomyomas arising from bilateral uterine remnants in a woman with MRKHS with coexisting DM1 is presented. Her chief complaint was abdominal distension. On pelvic magnetic resonance imaging (MRI), two solid pelvic masses showing low signal intensity on T2-weighted imaging were seen. Both the uterine corpus and cervix were unclear, but bilateral ovaries were observed normally on MRI. Two uterine leiomyoma-like masses connected by a band of fibrous tissue were found by laparotomy. As with the MRI findings, the uterine cervix and vagina could not be detected macroscopically. Normal bilateral adnexa and round ligaments were identified. All of her symptoms improved after hysterectomy.
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spelling doaj.art-40362fd87b27441a9e3cea337f9d37352024-11-02T23:53:20ZengHindawi LimitedCase Reports in Obstetrics and Gynecology2090-66922023-01-01202310.1155/2023/5182889Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature ReviewYukihiro Azuma0Koji Yamamoto1Mei Matsumoto2Hiroki Nagata3Ikumi Wada4Keisuke Miyamoto5Fuminori Taniguchi6Department of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyDepartment of Obstetrics and GynecologyMayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly of the genital tract. Since the secretion of sex hormones from the ovaries is preserved, leiomyomas and adenomyomas, which are estrogen-dependent diseases, may develop from the uterine remnant. In contrast, patients with myotonic dystrophy type 1 (DM1), the most common dystrophy in adults, are considered to be at high risk for benign tumors of the female reproductive system, such as uterine leiomyomas and ovarian cysts. A rare case of huge leiomyomas arising from bilateral uterine remnants in a woman with MRKHS with coexisting DM1 is presented. Her chief complaint was abdominal distension. On pelvic magnetic resonance imaging (MRI), two solid pelvic masses showing low signal intensity on T2-weighted imaging were seen. Both the uterine corpus and cervix were unclear, but bilateral ovaries were observed normally on MRI. Two uterine leiomyoma-like masses connected by a band of fibrous tissue were found by laparotomy. As with the MRI findings, the uterine cervix and vagina could not be detected macroscopically. Normal bilateral adnexa and round ligaments were identified. All of her symptoms improved after hysterectomy.http://dx.doi.org/10.1155/2023/5182889
spellingShingle Yukihiro Azuma
Koji Yamamoto
Mei Matsumoto
Hiroki Nagata
Ikumi Wada
Keisuke Miyamoto
Fuminori Taniguchi
Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
Case Reports in Obstetrics and Gynecology
title Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
title_full Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
title_fullStr Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
title_full_unstemmed Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
title_short Huge Leiomyomas Arising from Bilateral Uterine Remnants in a Mayer–Rokitansky-Küster-Hauser Syndrome Patient with Coexisting Myotonic Dystrophy Type 1: A Case Report and Literature Review
title_sort huge leiomyomas arising from bilateral uterine remnants in a mayer rokitansky kuster hauser syndrome patient with coexisting myotonic dystrophy type 1 a case report and literature review
url http://dx.doi.org/10.1155/2023/5182889
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