假性肌源性血管内皮瘤表现为阴茎病变。

IF 3.4 3区 医学 Q1 PATHOLOGY Virchows Archiv Pub Date : 2024-10-21 DOI:10.1007/s00428-024-03944-z
Reem Youssef, Jessica L Davis, William J Anderson, Andres M Acosta
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引用次数: 0

摘要

假性肌源性血管内皮瘤(PHE)是一种罕见的多灶性肿瘤,通常发生在患者生命的第二至第四个十年,男性居多。它通常发生在肢体远端,其特点是累及多个组织平面。这种肿瘤以阴茎原发病变的形式出现极为罕见,此前仅有五例文献记载。我们报告了另外五例表现为原发性阴茎肿瘤的 PHE 的临床病理特征,并回顾了以前发表的病例。这些肿瘤影响着年轻到中年的成年患者,临床表现相对平淡,类似于表皮包涵囊肿等不太活跃的病变。显微镜下,它们是由丰满的纺锤形细胞和圆形肿瘤细胞组成的界限不清的结节,这些细胞具有丰富、浓密的嗜酸性胞质和类似横纹肌母细胞的偏心核。肿瘤细胞呈浸润性生长,包括神经周围侵袭灶。免疫组化显示,角蛋白、内皮标志物(CD31和/或ERG)和FOSB始终共同表达。总之,阴茎PHE虽然罕见,但在鉴别诊断具有纺锤形细胞和/或横纹肌母细胞样形态的阴茎病变时,应考虑到它对中青年成年患者的影响。
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Pseudomyogenic hemangioendothelioma presenting as a penile lesion.

Pseudomyogenic hemangioendothelioma (PHE) is a rare, usually multifocal neoplasm typically affecting individuals in the second-to-fourth decade of life, with a male predominance. It often arises in the distal extremities and characteristically involves multiple tissue planes. Presentation of this neoplasm as a primary penile lesion is exceedingly rare, with only five cases previously documented in the literature. We report the clinicopathologic features of five additional PHEs presenting as primary penile tumors and review previously published cases. Tumors affected young to middle-aged adult patients and had a relatively bland clinical appearance, mimicking indolent lesions such as epidermal inclusion cysts. Microscopically, they were ill-defined nodules composed of plump spindle cells and round neoplastic cells with abundant, densely eosinophilic cytoplasm and eccentric nuclei resembling rhabdomyoblasts. Neoplastic cells demonstrated infiltrative growth, including foci of perineural invasion. Immunohistochemistry demonstrated invariable co-expression of keratins, endothelial markers (CD31 and/or ERG), and FOSB. In conclusion, penile PHE is rare but should be considered in the differential diagnosis of penile lesions with spindle cell and/or rhabdomyoblast-like morphology affecting young to middle-aged adult patients.

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来源期刊
Virchows Archiv
Virchows Archiv 医学-病理学
CiteScore
7.40
自引率
2.90%
发文量
204
审稿时长
4-8 weeks
期刊介绍: Manuscripts of original studies reinforcing the evidence base of modern diagnostic pathology, using immunocytochemical, molecular and ultrastructural techniques, will be welcomed. In addition, papers on critical evaluation of diagnostic criteria but also broadsheets and guidelines with a solid evidence base will be considered. Consideration will also be given to reports of work in other fields relevant to the understanding of human pathology as well as manuscripts on the application of new methods and techniques in pathology. Submission of purely experimental articles is discouraged but manuscripts on experimental work applicable to diagnostic pathology are welcomed. Biomarker studies are welcomed but need to abide by strict rules (e.g. REMARK) of adequate sample size and relevant marker choice. Single marker studies on limited patient series without validated application will as a rule not be considered. Case reports will only be considered when they provide substantial new information with an impact on understanding disease or diagnostic practice.
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