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[下腔静脉及右心房静脉内平滑肌瘤病。附1例报告并文献复习]

[Intravenous leiomyomatosis of the inferior vena cava and the right heart atrium. Apropos of a case and review of the literature].

作者信息

Alame A, Solovei G, Canal G, Petit J, Glavier F, Cart P, Leflon M, Karp P

机构信息

Hôpital Manchester, Charleville-Mézières.

出版信息

Ann Chir. 1993;47(3):270-8.

PMID:8333725
Abstract

The authors report a case of intravenous leiomyomatosis, a rare uterine tumor, extending to the inferior vena cava and to the right atrium. It seems to be the first case described in France. The wrong diagnosis of massive pulmonary embolism and the distance from a cardiac surgery unit led to emergency tumorectomy without cardiopulmonary bypass. A review of the literature studied the 24 cases with cardiac extension already reported, 19 operated and 5 autopsy reports. Right cardiac failure or syncopes are the most frequent clinical signs. Sometimes histologic examination after hysterectomy leads to the diagnosis. Echocardiography diagnoses an intra-atrial mass. Abdominal ultrasonography and phlebocavography show the iliocaval portion of the tumor. Cardiac angiography and computed tomography are also contributive. Surgical treatment except in extreme conditions should be performed by a cardiac surgical team. If the diagnosis of intra-venoux leiomyomatosis has been made preoperatively a one-stage cardiac and abdominal treatment should be preferred. In the other cases, cardiac surgery should be done first, allowing a precise histologic diagnosis and subsequent treatment of the iliocaval and uterine lesions.

摘要

作者报告了一例静脉内平滑肌瘤病,这是一种罕见的子宫肿瘤,已延伸至下腔静脉和右心房。这似乎是法国描述的首例病例。由于对大面积肺栓塞的误诊以及与心脏外科病房的距离,导致在没有体外循环的情况下进行了急诊肿瘤切除术。文献回顾研究了已报道的24例伴有心脏延伸的病例,其中19例接受了手术,5例为尸检报告。右心衰竭或晕厥是最常见的临床症状。有时子宫切除术后的组织学检查可得出诊断。超声心动图可诊断心房内肿块。腹部超声检查和静脉腔造影可显示肿瘤的髂腔部分。心脏血管造影和计算机断层扫描也有帮助。除极端情况外,手术治疗应由心脏外科团队进行。如果术前已诊断为静脉内平滑肌瘤病,应首选一期心脏和腹部联合治疗。在其他情况下,应首先进行心脏手术,以便进行精确的组织学诊断,随后治疗髂腔和子宫病变。

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