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Sarcomatoid Peritoneal Mesothelioma: Clinicopathologic Correlation of 13 Cases.

腹膜肉瘤样间皮瘤:13例临床病理相关性研究

Abstract

Peritoneal mesothelioma is rare, and the sarcomatoid variant is more infrequent, with <30 cases reported to date in the literature. Several case series have described the morphologic features of sarcomatoid peritoneal mesothelioma (SPe); however, the clinicopathologic features are not well characterized. To our knowledge, this is the first large series reporting the clinicopathologic features of SPe. We reviewed our database of 3106 malignant mesothelioma cases. Of 248 peritoneal mesotheliomas, 15 (4%) were sarcomatoid variant (0.5% of all mesotheliomas). Only cases with 100% sarcomatoid morphology diagnosed by open surgical biopsy and/or autopsy were included. Thus, 4 cases were excluded leaving 11 cases of SPe. Two additional cases of SPe previously published by 1 of the authors (V.L.R.), not included in the database, are added yielding 13 cases total. The median age at diagnosis was 66 years (range=48 to 85 y), and there was a male predominance (M:F=3.25:1). Survival from date of diagnosis to date of death was 5 months (range=0 to 12 mo). The most common presenting symptom was abdominal pain, and 3 of 4 women were suspected to have cholecystitis/cholelithiasis. All cases stained positive for cytokeratins, and 2 contained heterologous elements. Seven cases had objective markers of asbestos exposure, and 2 additional cases had occupations strongly associated with mesothelioma. Two cases with alleged household contact exposures could not be confirmed to be asbestos related by lung fiber analysis. SPe is a rare variant of mesothelioma attributed to asbestos exposure in 69% of our cases.

摘要

腹膜间皮瘤少见,肉瘤样亚型更不常见,目前文献报道不足30例。几个病例系列报道描述了腹膜肉瘤样间皮瘤(SPe)的形态学特征,但其临床病理特征缺乏详细描述。 据我们所知,本文是关于SPe临床病理特征的首次大宗病例系列报道。我们复习了3106例恶性间皮瘤,其中腹膜间皮瘤248例,15例为肉瘤样亚型(占腹膜间皮瘤的4%,占所有间皮瘤的0.5%)。从15例SPe中按照形态学全部呈肉瘤样表现、通过手术活检和/或尸检标本病理确诊作为纳入标准,排除掉4例,剩下11例SPe。另外2例,不包含在资料库中作者之一(V.L.R.)以前发表过报道,纳入研究,共计13例SPe。病人中位发病年龄66岁(年龄范围48—85岁),男性为主(男:女=3.25:1)。从确诊到死亡,生存期为5个月(范围0—12个月)。最常见的临床症状为腹痛,4例女性病人中有3例怀疑胆囊炎/胆石症。全部病例细胞角蛋白阳性,其中2例含异源性成分。7名病人用相应的客观指标证明有石棉接触史,另纳入的2名病人职业与间皮瘤有很强的相关性。有所谓家庭接触史的2例,通过肺纤维分析,证实与石棉不相关。SPe是一种少见的间皮瘤,我们的研究显示69%的病例归因于石棉接触史。

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