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 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.

Source: Neuropathology - April 1, 2017 Category: Neurology Authors: Magda Zanelli, Moira Ragazzi, Giovanni Marchetti, Alessandra Bisagni, Massimo Principi, Daniela Fanni, Elisabetta Froio, Silvia Serra, Eleonora Zanetti, Loredana De Marco, Felice Giangaspero, Stefano Ascani Tags: Case Report Source Type: research