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表现为弥漫性软脑膜疾病的原发性组织细胞肉瘤：病例描述及文献复习

Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature.

作者信息

Zanelli Magda, Ragazzi Moira, Marchetti Giovanni, Bisagni Alessandra, Principi Massimo, Fanni Daniela, Froio Elisabetta, Serra Silvia, Zanetti Eleonora, De Marco Loredana, Giangaspero Felice, Ascani Stefano

机构信息

Pathology Unit, Arcispedale Santa Maria Nuova - IRCCS, Reggio Emilia, Italy.

Institute of Pathology, Ospedale di Terni, University of Perugia, Italy.

出版信息

Neuropathology. 2017 Dec;37(6):517-525. doi: 10.1111/neup.12390. Epub 2017 May 26.

DOI:10.1111/neup.12390
PMID:28548244
Abstract

Histiocytic sarcoma is a rare malignant neoplasm arising most commonly in lymph nodes, intestinal tract, skin and soft tissue. The incidence of primary CNS histiocytic sarcoma is even rarer with a total of just 27 cases reported in the literature so far. Herein we describe the first autopsy case of histiocytic sarcoma presenting as a diffuse leptomeningeal disease in absence of a CNS tumor-forming parenchymal lesion. The clinical, pathological and immunophenotypic features are described and an updated literature review on primary CNS histiocytic sarcoma is included.

摘要

组织细胞肉瘤是一种罕见的恶性肿瘤，最常发生于淋巴结、肠道、皮肤和软组织。原发性中枢神经系统组织细胞肉瘤的发病率更为罕见，迄今为止文献中总共仅报道了27例。在此，我们描述了首例组织细胞肉瘤尸检病例，该病例表现为弥漫性软脑膜疾病，而无中枢神经系统形成肿瘤的实质病变。本文描述了其临床、病理和免疫表型特征，并纳入了对原发性中枢神经系统组织细胞肉瘤的最新文献综述。

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