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

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

Neuropathology. 2017 May 26;:

Authors: Zanelli M, Ragazzi M, Marchetti G, Bisagni A, Principi M, Fanni D, Froio E, Serra S, Zanetti E, De Marco L, Giangaspero F, Ascani S

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.

PMID: 28548244 [PubMed - as supplied by publisher]

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