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软组织多形性透明变性血管扩张性肿瘤:1例报告并文献复习

Pleomorphic hyalinizing angiectatic tumour of soft parts: a case report and review of the literature.

作者信息

Jaggon J R, Aitken R D C

机构信息

Department of Pathology, The University of the West Indies, Kingston 7, Jamaica, West Indies.

出版信息

West Indian Med J. 2007 Dec;56(6):544-6.

Abstract

Pleomorphic hyalinizing angiectatic tumour (PHAT) is a recently described, rare, low-grade soft tissue neoplasm. The lesion is characterized by clusters of hyalinized and thrombosed ectatic vessels alternating with a variably cellular stroma composed of atypical cells, many with intranuclear pseudoinclusions. Other features are inflammatory cell infiltration, haemosiderin deposits, focal calcification and minimal to absent mitoses. No metastases have so far been described; however the local recurrence rate has been found to be high. To date, approximately 60 such cases of PHAT and its precursor, "early PHAT", have been described in the world literature. We report the first known case of PHAT from this institution which occurred in the left loin of a 77-year old woman. Three years previously, a smaller lesion excised from the same location had been called an ancient schwannoma on histology. This is the most common differential diagnosis offered for this entity even though the two differ in immunohistochemical profile. 'Early PHAT' was also identified on the periphery of the recurrent lesion.

摘要

多形性透明变性血管扩张性肿瘤(PHAT)是一种最近才被描述的罕见的低度软组织肿瘤。该病变的特征是透明变性和血栓形成的扩张血管簇与由非典型细胞组成的细胞成分各异的间质交替存在,许多非典型细胞有核内假包涵体。其他特征包括炎症细胞浸润、含铁血黄素沉积、局灶性钙化以及极少或无核分裂象。目前尚未见有转移的报道;然而,局部复发率较高。迄今为止,世界文献中已报道了约60例此类PHAT及其前驱病变“早期PHAT”。我们报告了本机构首例已知的PHAT病例,发生于一名77岁女性的左腰部。三年前,从同一部位切除的一个较小病变在组织学上被诊断为陈旧性神经鞘瘤。尽管两者在免疫组化特征上有所不同,但这是该实体最常见的鉴别诊断。在复发病变的周边也发现了“早期PHAT”。

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