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

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.

2017-01-01

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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.