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原发性肺低度纤维黏液样肉瘤:一例诊断难题的罕见病例。

Primary lung low-grade fibromyxoid sarcoma: A rare case with A diagnostic dilemma.

作者信息

Ayadi Aida, Houcine Yoldez, Moussa Chirine, Rouis Houda, Abid Tarek, Hugues Begueret, Le Loarer François

机构信息

Faculty of Medicine, El Manar University, Ariana, Tunisia.

Pneumology Department 1, Abderrahmen Mami Hospital, Ariana, Tunisia.

出版信息

Rare Tumors. 2024 Feb 29;16:20363613241234201. doi: 10.1177/20363613241234201. eCollection 2024.

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) is a rare, low-grade malignant soft tissue tumor that is often mistaken for benign or more rarely malignant tumor types. Commonly, this tumor affects young adults and typically arises in the deep proximal extremities or trunk with frequent recurrences and can metastasize to the lungs many years late. Visceral LGFMS is extremely rare. Only a few cases of primary LGFMS of the lung have been reported. Here, we present the clinical, gross, microscopic, and immunohistochemical characteristics of Evans tumor occurring in the lung with a review of the literature and discuss the differential diagnosis in this exceptional localization.

摘要

低度纤维黏液样肉瘤(LGFMS)是一种罕见的低度恶性软组织肿瘤,常被误诊为良性肿瘤,或更罕见地被误诊为其他恶性肿瘤类型。通常,这种肿瘤好发于年轻人,典型部位是四肢近端深部或躯干,易复发,多年后可转移至肺部。内脏型LGFMS极为罕见。仅报道过少数原发性肺LGFMS病例。在此,我们呈现发生于肺的埃文斯肿瘤的临床、大体、显微镜及免疫组化特征,并复习文献,讨论这一特殊部位的鉴别诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/761c/10906048/0dc85c52e6c2/10.1177_20363613241234201-fig1.jpg

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