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良性转移性平滑肌瘤所致右心衰竭:1例极其罕见病例报告及文献复习

Right heart failure due to benign metastasizing leiomyoma: a case report of this exceedingly rare condition and review of the literature.

作者信息

Pedrosa Cesar, Miotto Heberth Cesar, Drumond Leonardo Ferber, Andrade Mariana Magalhães, Zille Paula Chaves, Palhares Gisele Tubertini, Abrahao-Machado Lucas Faria

机构信息

Biocor Instituto, Alameda Oscar Niemeyer, 217, Nova LIma, MG, Brazil, 34006-056.

Bacchi Lab, Rua Major Leonidas Cardoso, 739, Botucatu, São Paulo, Brazil, 18602-010.

出版信息

Eur Heart J Case Rep. 2020 Dec 10;4(6):1-7. doi: 10.1093/ehjcr/ytaa468. eCollection 2020 Dec.

Abstract

BACKGROUND

Benign metastasizing leiomyoma (BML) is a smooth muscle tumour of genital origin occurring in women with a history of uterine or pelvic leiomyoma. Although histologically benign, it exhibits metastatic behaviour. Lungs are the most common site of metastasis. The heart is a rare site and metastasis at this location has been described in just few cases.

CASE SUMMARY

A 42-year-old woman with a resected uterine leiomyoma and a subsequent and still not-resected left periovarian solid mass began complaining of shortness of breath 2 weeks before presentation. Echocardiography showed a mass located in the right ventricular cavity, enlargement of the right ventricle, and severe tricuspid regurgitation. Cardiac magnetic resonance revealed two masses suggestive of tumours in the right ventricle causing right ventricular outflow tract obstruction. Cardiac surgery was performed and, intraoperatively, a third small mass was detected on the tricuspid valve. The masses were resected, and tricuspid valve replacement was performed. Ten days later, the patient underwent an abdominal surgery for the pelvic mass resection. Immunohistochemical analysis of the cardiac and pelvic masses corroborated the diagnosis of benign leiomyomas. The patient was discharged in good clinical condition.

DISCUSSION

Benign metastasizing leiomyoma to the heart is a rare condition. The pathogenesis remains controversial and includes: (i) vascular or lymphatic spread of myomatous tissue cells when leiomyoma resection or hysterectomy is performed and (ii) smooth muscle cell proliferation in multiple regions. The more usual locations of BML in the heart seem to be the tricuspid valve and the right face of the interventricular septum.

摘要

背景

良性转移性平滑肌瘤(BML)是一种起源于生殖器的平滑肌肿瘤,发生于有子宫或盆腔平滑肌瘤病史的女性。尽管组织学上为良性,但它表现出转移行为。肺是最常见的转移部位。心脏是罕见的转移部位,仅在少数病例中有此部位转移的描述。

病例摘要

一名42岁女性,曾行子宫平滑肌瘤切除术,随后出现左侧卵巢旁实性肿块且尚未切除,在就诊前2周开始出现气短症状。超声心动图显示右心室腔内有一肿块,右心室扩大,伴有严重三尖瓣反流。心脏磁共振成像显示右心室内有两个提示肿瘤的肿块,导致右心室流出道梗阻。进行了心脏手术,术中在三尖瓣上发现了第三个小肿块。切除了肿块,并进行了三尖瓣置换术。十天后,患者接受了腹部手术以切除盆腔肿块。对心脏和盆腔肿块的免疫组织化学分析证实为良性平滑肌瘤。患者出院时临床状况良好。

讨论

心脏良性转移性平滑肌瘤是一种罕见疾病。其发病机制仍存在争议,包括:(i)在进行平滑肌瘤切除术或子宫切除术时肌瘤组织细胞的血管或淋巴扩散,以及(ii)多个区域的平滑肌细胞增殖。BML在心脏中较常见的部位似乎是三尖瓣和室间隔右缘。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2462/7793117/21b2b89a16c8/ytaa468f1.jpg

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本文引用的文献

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Suspicious Right Heart Mass: A Rare Case of Benign Metastasizing Leiomyoma of the Tricuspid Valve.
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