Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature. Issue 6 (26th May 2017)

• Record Type: Journal Article
• 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
• Is Part Of: Neuropathology. Volume 37:Issue 6(2017:Dec.)
• Page Start: 517
• Page End: 525
• Publication Date: 2017-05-26
• Subjects: brain -- central nervous system -- histiocytic sarcoma -- malignant histiocytosis -- meninges; Nervous system -- Diseases -- Periodicals; Nervous system -- Pathophysiology -- Periodicals; 616.8047
• DOI: 10.1111/neup.12390
• Languages: English
• ISSNs: 0919-6544

Abstract

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.