Persistent marked cerebrospinal fluid eosinophilia in a dog with primary central nervous system histiocytic sarcoma

A 6-year-old female spayed Jack Russell Terrier was evaluated for episodic seizure-like activity and intermittent obtundation over the previous 3 weeks. Magnetic resonance imaging (MRI) of the brain revealed mild generalized dilation of the ventricular system with periventricular edema. A focal area of mildly increased lepto- and pachymeningeal contrast uptake in the region of the right parietal and occipital lobes was observed. Analysis of cerebrospinal fluid (CSF) revealed marked mixed pleocytosis with 20% eosinophils and no atypical cells or microorganisms. The dog transiently improved with prednisolone for suspected eosinophilic meningoencephalitis/meningoencephalomyelitis of unknown origin (MUO) but worsened over the following 5 months. Brain MRI and CSF sampling were repeated. Additional multifocal lesions were evident in the brainstem and cerebellum. On CSF analysis, the eosinophilic pleocytosis and increased total protein persisted. The clinical signs progressed despite treatment, and the patient was euthanized 6 weeks later. A post-mortem examination was performed. Histopathology and immunohistochemistry revealed a multifocal neoplastic proliferation of cells in the brain, diffusely and strongly positive for ionized calcium-binding adapter molecule (Iba-1) and negative for AE1/AE3 pan-cytokeratin and glial-fibrillar-acid-protein (GFAP) immunostaining, consistent with a diagnosis of histiocytic sarcoma (HS). No other organic lesions were found; therefore, the neoplasm was considered a primary HS of the central nervous system (CNS). This case report stresses the importance of considering primary CNS HS in the differential diagnosis of dogs with marked CSF eosinophilia, even in the absence of atypical cells on cytologic examination.