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

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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
JournalNeuropathology
DOIs
Publication statusAccepted/In press - 2017

Keywords

  • Brain
  • Central nervous system
  • Histiocytic sarcoma
  • Malignant histiocytosis
  • Meninges

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature'. Together they form a unique fingerprint.

Cite this