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腹腔镜干预梅克尔-罗基坦斯基-库斯特-豪泽综合征中的盆腔实体肿瘤:一例先天性残角子宫双侧子宫腺肌瘤。

Laparoscopic intervention for solid pelvic tumours in Mayer-Rokitansky-Küster-Hauser syndrome: a case of bilateral uterine adenomyomas of the rudimentary uterus.

机构信息

Obstetrics and Gynecology, Seirei Hamamatsu Hospital, Hamamatsu, Shizuoka, Japan

Obstetrics and Gynecology, Seirei Hamamatsu Hospital, Hamamatsu, Shizuoka, Japan.

出版信息

BMJ Case Rep. 2023 May 8;16(5):e254262. doi: 10.1136/bcr-2022-254262.

Abstract

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a condition caused by Müllerian anomalies, is characterised by congenital vaginal aplasia and a rudimentary uterus. Case reports concerning uterine fibroids associated with MRKH syndrome are limited, and differentiating between uterine fibroids and ovarian solid tumours prior to surgical intervention is often challenging. Here, we present the case of a patient with MRKH syndrome and asymptomatic bilateral pelvic solid tumours located close to both ovaries. Based on intraoperative and histopathological findings, the tumours were diagnosed as adenomyomas of the rudimentary uterus. This is the first reported case of a uterine adenomyoma associated with MRKH syndrome. Moreover, our report highlights the fact that diagnostic laparoscopy is a valuable method to evaluate pelvic tumours in MRKH syndrome.

摘要

Mayer-Rokitansky-Küster-Hauser (MRKH) 综合征是一种由 Müllerian 异常引起的疾病,其特征为先天性阴道发育不全和子宫发育不良。与 MRKH 综合征相关的子宫肌瘤病例报告有限,且在手术干预前区分子宫肌瘤和卵巢实性肿瘤通常具有挑战性。在这里,我们报告了一例 MRKH 综合征患者,其无症状的双侧盆腔实性肿瘤靠近双侧卵巢。基于术中及组织病理学检查结果,诊断为子宫残角的腺肌瘤。这是首例报道的与 MRKH 综合征相关的子宫腺肌瘤病例。此外,我们的报告强调了诊断性腹腔镜检查是评估 MRKH 综合征中盆腔肿瘤的一种有价值的方法。

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