• William Clifton, Oluwaseun Oluwadara Akinduro, Sebastian Lopez-Chiriboga, Dale Alan Whitaker, and Ronald Reimer.
• Department of Neurological Surgery, Mayo Clinic Florida, Jacksonville, Florida, USA. Electronic address: clifton.william@mayo.edu.
• World Neurosurg. 2017 Oct 1; 106: 1053.e1-1053.e5.

BackgroundPrimary central nervous system (CNS) histiocytic sarcoma is an extremely rare lymphoproliferative disorder that affects the CNS and behaves aggressively. Only 27 cases of primary CNS histiocytic sarcoma have been reported. The paucity of literature on this entity has made diagnosis and treatment difficult both for the surgeon and the pathologist.Case DescriptionIn this case of primary CNS histiocytic sarcoma, a middle-aged woman presented from an outside institution with a supposed cerebellar abscess. Intraoperative frozen pathology was initially interpreted as high-grade glioma; however, final pathology demonstrated histiocytic sarcoma.ConclusionsThis report makes a significant contribution to the literature on this rare malignant disease by outlining a similar presentation among several cases and providing a thorough overview of existing criteria for diagnosis and management.Copyright © 2017 Elsevier Inc. All rights reserved.

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