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

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...

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Bibliographic Details
Published in:Neuropathology 2017-12, Vol.37 (6), p.517-525
Main Authors: 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
Format: Article
Language:English
Subjects:
Autopsy
brain
Central nervous system
histiocytic sarcoma
Intestine
Literature reviews
Lymph nodes
malignant histiocytosis
Meninges
Neoplasia
Sarcoma
Skin
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Description
Summary: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.
ISSN:0919-6544
1440-1789
DOI:10.1111/neup.12390