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Título

Primary central nervous system lymphoma: the challenges of diagnostic workup - Case report

RESUMO

CASE PRESENTATION: A 16-year-old male patient developed hypersomnia, dysarthria, left hemiparesis, loss of sphincter control and seizures for 4 months. He received acyclovir, followed by pulse therapy with methylprednisolone, showing clinical improvement. After 2 months, he evolved with cognitive deficit, axial and appendicular ataxia, and diparesis. He received another pulse therapy, with no response and showed progressive impairment of ambulation. During the Immunoglobulin infusion, he developed clinical complications. Under intensive care, he performed plasmapheresis, with no improvement. Autoimmune markers were negative. The search for neoplastic cells in the cerebrospinal fluid (CSF) was inconclusive. Infectious screening was negative, as was extraneural neoplastic screening. Spinal resonance was normal and brain resonance showed multiple supra and infratentorial lesions, with moderate mass effect, post-contrast enhancement and moderate water diffusion restriction, with a nodular lesion in the pineal; proton spectroscopy suggested cell proliferation. Brain biopsy favored the diagnosis of lymphoproliferative disease. Immunohistochemistry described high-grade B-cell lymphoma of the central nervous system. With the diagnostic confirmation, she underwent systemic chemotherapy, showing slight clinical and radiological improvement, but it was interrupted by infectious complications, which led to death. DISCUSSION: Primary central nervous system lymphoma is a rare and aggressive non-Hodgkin lymphoma that may involve the brain, leptomeninges, spinal cord or eyes. Approximately 90% are of the diffuse large B-cell lymphoma type. Congenital or acquired immunodeficiency is the main risk factor. In immunocompetent patients, the mean age at diagnosis is 53 to 57 years. Neurological symptoms tend to overlap with systemic ones. Diagnosis requires neuroimaging, CSF analysis and brain biopsy. Treatment involves radiotherapy and chemotherapy. FINAL COMMENTS: The purpose of this report is to describe a case of Primary central nervous system lymphoma, a rare disease in a young and immunocompetent patient, emphasizing the importance of its recognition among the differential diagnoses, and the consequent search for timely therapy, providing greater survival.

Palavras Chave

Primary central nervous system lymphoma; young; immunocompetent; diagnostic