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Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature
Author(s) -
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
Publication year - 2017
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12390
Subject(s) - histiocytic sarcoma , medicine , pathology , histiocyte , sarcoma , pathological , autopsy , lymph , lesion , soft tissue sarcoma
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.