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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.

DOI:10.1136/bcr-2022-254262
PMID:37156565
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10173994/
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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Laparoscopic intervention for solid pelvic tumours in Mayer-Rokitansky-Küster-Hauser syndrome: a case of bilateral uterine adenomyomas of the rudimentary uterus.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。
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J Pediatr Adolesc Gynecol. 2013 Aug;26(4):224-7. doi: 10.1016/j.jpag.2013.03.004.
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J Minim Invasive Gynecol. 2015 Jan;22(1):142-50. doi: 10.1016/j.jmig.2014.09.003. Epub 2014 Sep 6.
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本文引用的文献

1
The Rare, Unexpected Condition of a Twisted Leiomyoma in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Etiopathogenesis, Diagnosis and Management. Our Experience and Narrative Review of the Literature.梅耶-罗基坦斯基-库斯特-豪泽综合征(MRKH)中罕见且意外的扭转平滑肌瘤情况:病因病机、诊断与处理。我们的经验及文献叙述性综述
Int J Environ Res Public Health. 2021 May 31;18(11):5895. doi: 10.3390/ijerph18115895.
2
A rare case of multiple leiomyomas on rudimentary uterus in a woman with Mayer Rokitansky Kuster Hauser (MRKH) syndrome: A challenging diagnosis and laparoscopic approach.一名患有迈耶-罗基坦斯基-库斯特-豪泽综合征(MRKH综合征)的女性残角子宫多发平滑肌瘤罕见病例:具有挑战性的诊断及腹腔镜手术方法
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3
Extrauterine adenomyoma: a review of the literature.子宫外腺肌症:文献回顾。
Eur J Obstet Gynecol Reprod Biol. 2018 Sep;228:130-136. doi: 10.1016/j.ejogrb.2018.06.021. Epub 2018 Jun 12.
4
Diagnosis and Management of Ovarian Tumor in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome.Mayer-Rokitansky-Küster-Hauser(MRKH)综合征中的卵巢肿瘤的诊断与治疗。
Biomed Res Int. 2018 Mar 12;2018:2369430. doi: 10.1155/2018/2369430. eCollection 2018.
5
Two sisters with Mayer-Rokitansky-Küster-Hauser syndrome and serous adenocarcinoma of the ovary.两名患有迈耶-罗基坦斯基-库斯特-豪泽综合征及卵巢浆液性腺癌的姐妹。
Gynecol Oncol Rep. 2017 Sep 7;22:13-15. doi: 10.1016/j.gore.2017.09.002. eCollection 2017 Nov.
6
Mayer-Rokitansky-Küster-Hauser Syndrome With Bilateral Ovarian Sertoli Cell Tumors: Review of the Literature and Report of a Rare Case.伴有双侧卵巢支持细胞瘤的梅耶-罗基坦斯基-库斯特-豪泽综合征:文献综述及1例罕见病例报告
Female Pelvic Med Reconstr Surg. 2018 Sep/Oct;24(5):e32-e34. doi: 10.1097/SPV.0000000000000483.
7
Spectrum of MRI Appearance of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in Primary Amenorrhea Patients.原发性闭经患者中迈耶-罗基坦斯基-库斯特-豪泽综合征(MRKH)的MRI表现谱
J Clin Diagn Res. 2017 Jul;11(7):TC30-TC35. doi: 10.7860/JCDR/2017/29016.10317. Epub 2017 Jul 1.
8
Rare Case of Leiomyoma and Adenomyosis in Mayer-Rokitansky-Kuster-Hauser Syndrome.迈耶-罗基坦斯基-库斯特-豪泽综合征合并平滑肌瘤和子宫腺肌病的罕见病例
Case Rep Obstet Gynecol. 2016;2016:3725043. doi: 10.1155/2016/3725043. Epub 2016 Oct 23.
9
Leiomyoma Arising from Mullerian Remnant, Mimicking Ovarian Tumor in a Woman with MRKH Syndrome and Unilateral Renal Agenesis.起源于苗勒管残余组织的平滑肌瘤,在一名患有苗勒管发育不全综合征和单侧肾缺如的女性中酷似卵巢肿瘤。
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A rare case of Mayer-Rokitansky-Kuster-Hauser syndrome with multiple leiomyomas in hypoplastic uterus.一例罕见的苗勒管发育不全综合征合并子宫发育不全伴多发平滑肌瘤。
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