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[乳头肌纤维弹性瘤的临床诊断]

[Clinical diagnostics of papillary fibroelastoma].

作者信息

Trisvetova E L

出版信息

Klin Med (Mosk). 2007;85(12):7-11.

PMID:18318158
Abstract

Papillary fibroelastoma (PFE) is a benign heart tumor, the diagnosis of which is difficult. Clinical manifestations of PFE are non-specific and scarce, and for this reason the tumor is often revealed during a heart surgery of an autopsy. PFE affects patients of all ages from neonates to 96-year-old ones and is often localized on valvular cusps, although vegetations may appear on other intracardiac structures. The histogenesis of the tumor is unclear; there are several theories of PFE origin (hemodynamic effects on the myocardium, viral theory, iatrogenic theory etc.) Macroscopically the tumor is small size, villous, whitish-grey, consists of a pedicle and villi of gely-like soft or dense-elastic consistence. The mobility of the tumor and the fragmentation of its tissue lead to its main complications such as obstruction of cardiac cameras (in-flow and out-flow disorders) and embolic syndrome in various basins (the brain, coronary arteries, the eye, the kidneys, and the lungs). The diagnosis of PFE is made using EchoCG, preferably transesophageal one. Treatment of PFE is surgical and consists of tumor or cusp removal. PFE should be differentiated from other benign and malignant heart tumors, infective endocarditis, heart echinococcosis, coronary artery disease, and cerebrovascular diseases.

摘要

乳头状纤维弹性瘤(PFE)是一种良性心脏肿瘤,其诊断较为困难。PFE的临床表现不具特异性且少见,因此该肿瘤常在尸检心脏手术期间被发现。PFE可累及从新生儿到96岁的各年龄段患者,常位于瓣膜尖,不过赘生物也可能出现在其他心内结构上。肿瘤的组织发生尚不清楚;关于PFE的起源有几种理论(对心肌的血流动力学影响、病毒理论、医源性理论等)。从宏观上看,肿瘤体积小,呈绒毛状,灰白色,由蒂和凝胶样软或致密弹性的绒毛组成。肿瘤的活动及其组织的破碎导致其主要并发症,如心腔阻塞(流入和流出障碍)以及不同部位(脑、冠状动脉、眼、肾和肺)的栓塞综合征。PFE的诊断采用超声心动图,最好是经食管超声心动图。PFE的治疗是手术治疗,包括切除肿瘤或瓣膜尖。PFE应与其他良性和恶性心脏肿瘤、感染性心内膜炎、心脏棘球蚴病、冠状动脉疾病和脑血管疾病相鉴别。

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引用本文的文献

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Presentation of papillary fibroelastoma of the aortic valve with atypical symptoms.主动脉瓣乳头状纤维弹性瘤伴非典型症状的表现。
BMJ Case Rep. 2018 Jul 19;2018:bcr-2018-224367. doi: 10.1136/bcr-2018-224367.