A rare neuromyelitis optica mimic: Primary CNS histiocytic sarcoma. (September 2022)

• Record Type: Journal Article
• Authors: Rogawski, David S; Nirschl, Jeffrey J; McDonald, Jamie; Nie, Esther; Schwartz, Nicholas U; Vogel, Hannes; Scott, Brian J; Gold, Carl A; Kipp, Lucas B
• Is Part Of: Multiple sclerosis. Volume 28:Number 10(2022)
• Page Start: 1651
• Page End: 1654
• Publication Date: 2022-09
• Subjects: Primary CNS histiocytic sarcoma -- NMOSD mimic -- case report; Central nervous system -- Diseases -- Periodicals; Myelin sheath -- Diseases -- Periodicals; Inflammation -- Periodicals; Multiple sclerosis -- Periodicals; Central Nervous System Diseases -- Periodicals; Demyelinating Diseases -- Periodicals; Inflammation -- Periodicals; Multiple Sclerosis -- Periodicals; Système nerveux central -- Maladies -- Périodiques; Gaine de myéline -- Maladies -- Périodiques; Inflammation (Pathologie) -- Périodiques; Sclérose en plaques -- Périodiques; Electronic journals; 616.834005
• DOI: 10.1177/13524585221097564
• Languages: English
• ISSNs: 1352-4585

Abstract

Primary central nervous system (CNS) histiocytic sarcoma is a rare hematolymphoid malignancy with features of mature histiocytes and carries a poor prognosis. We describe a unique case in which a 50-year-old woman presented with recurrent acute brainstem syndrome, area postrema syndrome, and myelitis with corresponding magnetic resonance imaging (MRI) lesions meeting diagnostic criteria for seronegative neuromyelitis optica spectrum disorder (NMOSD). Despite initial improvement with steroids and plasma exchange, she experienced recurrent symptoms over 10 months referable to new and persistently enhancing lesions. At autopsy, neuropathology revealed a diffusely infiltrative primary CNS histiocytic sarcoma. This case represents a rare clinicoradiologic mimic of NMOSD, underscoring the importance of evaluation for infiltrative diseases in cases of atypical seronegative NMOSD.