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.
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. - In: NEUROPATHOLOGY. - ISSN 1440-1789. - (2017). [10.1111/neup.12390]
Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature
Ragazzi M;
2017-01-01
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.
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