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多发性“花斑状”乳头状弹性纤维瘤。

Multifocal "tapete" papillary fibroelastoma.

机构信息

Department of Pathology, Toronto General Hospital/University Health Network, Toronto, Ontario M5G 2C4, Canada.

出版信息

J Clin Pathol. 2009 Dec;62(12):1066-70. doi: 10.1136/jcp.2009.070243.

Abstract

Papillary fibroelastomas (PFEs) are benign cardiac tumours that typically arise on diseased aortic or mitral valves. Usually solitary, PFEs are characterised by numerous frond-like branches attached to the valve surface by a single stalk, elastic tissue present within the central core, and the appearance of a sea anemone when immersed in water. PFEs have a predilection for men with a mean age of 56 years. A very rare case of a 25-year-old man with multiple lesions on the aortic and mitral valves and a large confluent "carpet-like" lesion in the left ventricular cavity is presented. Admitted with a significant left middle cerebral artery stroke and hemiplegia, investigations showed a left ventricular outflow tract lesion, with surgical excision displaying a multifocal "carpet-like" PFE. Histopathological examination showed various lesions presenting with a short stem, several fronds arising from each stem like a "tree trunk", and elastic tissue within the central core. This case is presented, along with a review of 833 reported cases of PFEs published after the year 2001. It is believed that this is the first reported instance of surgically resected and morphologically confirmed multiple PFEs seen and removed at one operation.

摘要

乳头状纤维弹性瘤 (PFEs) 是一种良性心脏肿瘤,通常发生在病变的主动脉瓣或二尖瓣上。PFEs 通常为单发,其特征为许多扇形分支通过单个柄附着在瓣膜表面,中央核心内存在弹性组织,在水中浸泡时呈现海葵的外观。PFEs 多见于男性,平均年龄为 56 岁。本文报道了一例非常罕见的 25 岁男性,其主动脉瓣和二尖瓣上有多个病变,左心室腔内有一个大的融合“地毯样”病变。该患者因左侧大脑中动脉卒中伴偏瘫入院,检查显示左心室流出道病变,手术切除显示多灶性“地毯样”PFEs。组织病理学检查显示各种病变,其特征为短柄,每个柄上有几个分支,类似于“树干”,中央核心内有弹性组织。本文报道了这一病例,并回顾了 2001 年后发表的 833 例 PFEs 报道病例。据信,这是首例经手术切除并经形态学证实的多发 PFEs 的病例,且在一次手术中切除。

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