Leiomyoma development in Mayer-Rokitansky-Küster-Hauser syndrome: a case report and a narrative review of the literature
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The development of leiomyomas on the grounds of an aplastic/hypoplastic uterus in patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) has been rarely described. We report the first case of development of multipleleiomyomas in a patient with MRKHS complicated with pulmonary valve stenosis, and we present a narrative reviewof the existing literature. A 44-year-old patient with MRKHS attended our clinic because of pelvic pain, whichwas attributed to a pelvic mass found on ultrasound. Magnetic resonance imaging revealed a multinodular mass,indicating either ovarian pathology or the presence of leiomyomas. Exploratory laparotomy was performed, andmultiple solid masses on the grounds of two rudimentary uterine buds were observed. Histological analysis revealedmultiple leiomyomas arising from parametrial or paratubal tissue. We searched medical databases for articles relevantto leiomyomas and MRKHS. We present a review of the current literature and summarize the clinical manifestation,diagnosis, management, and histopathological findings of all the cases described. We underline that it is importantfor gynecologists to be aware of this rare clinical entity, and symptomatic leiomyomas cannot be excluded in patientswith MRKHS.
Abstract
The development of leiomyomas on the grounds of an aplastic/hypoplastic uterus in patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) has been rarely described. We report the first case of development of multipleleiomyomas in a patient with MRKHS complicated with pulmonary valve stenosis, and we present a narrative reviewof the existing literature. A 44-year-old patient with MRKHS attended our clinic because of pelvic pain, whichwas attributed to a pelvic mass found on ultrasound. Magnetic resonance imaging revealed a multinodular mass,indicating either ovarian pathology or the presence of leiomyomas. Exploratory laparotomy was performed, andmultiple solid masses on the grounds of two rudimentary uterine buds were observed. Histological analysis revealedmultiple leiomyomas arising from parametrial or paratubal tissue. We searched medical databases for articles relevantto leiomyomas and MRKHS. We present a review of the current literature and summarize the clinical manifestation,diagnosis, management, and histopathological findings of all the cases described. We underline that it is importantfor gynecologists to be aware of this rare clinical entity, and symptomatic leiomyomas cannot be excluded in patientswith MRKHS.
참고문헌 (15)
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