Abstract
BackgroundCentral neurocytomas represent 0.25–0.5% of all intracranial tumors in adults. Leptomeningeal spread is uncommon, and the exact incidence of meningeal spread is unknown due to sparse literature. We present the clinical course and management outcome of a case of atypical central neurocytoma with leptomeningeal spread.Case presentationA young gentleman, who initially presented with memory loss, was found to have a right intra-axial periventricular mass on imaging. He underwent subtotal resection, and operative histopathology suggested a periventricular atypical neurocytoma. In view of subtotal resection, adjuvant focal radiation therapy was recommended, but he developed headache and blurring of vision 10 days postoperatively. Contrast enhanced craniospinal magnetic resonance imaging (MRI) showed residual primary tumor as well as diffuse leptomeningeal spread. Cerebrospinal fluid cytology also showed malignant cells. After tumor board discussion, craniospinal axis irradiation was advised and delivered. He remained disease-free for 10 months after radiation therapy, but then developed local and spinal recurrence, and offered salvage chemotherapy. His general condition deteriorated following chemotherapy with disease progression, and he was subsequently advised best supportive care.ConclusionLeptomeningeal dissemination in atypical neurocytomas portends an aggressive course and adverse prognosis; management decisions may need tailoring as per individual presentation.
Highlights
Central neurocytomas represent 0.25–0.5% of all intracranial tumors in adults
Central neurocytomas (CN) represent 0.25–0.5% of all intracranial tumors in adults, mostly affecting patients in third decade and are even more uncommon in children [1]. In their first description of CN in 1982, Hassoun et al characterized two tumors that were neuronal on electron microscopy but resembled oligodendroglioma rather than medulloblastoma on light microscopy [2]
Computed tomography (CT) typically shows a ventricular space occupying lesion, usually involving lateral ventricles; the mass is well circumscribed with attachment to septum pellucidum and enhances with iodinated contrast
Summary
Atypical central neurocytoma may occasionally exhibit aggressive behavior. Treatment may need to be tailored to specific presentation, imaging, and pathologic findings.
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