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Título

Diffuse large B Cell Lymphoma with Secondary Central Nervous System with atypical presentation

RESUMO

Case presentation: A 67-year-old man presented with 20 days of mental confusion, emotional apathy, dysphagia, followed by decreased level of consciousness. Previously, he had a weight loss of 10 kg in 3 months. He had not fever, night sweats or headache, and had no relevant comorbidities. At examination, he had multiple cervical lymphadenopathy, spontaneous eye opening, localized painful stimulus, no verbal response. MRC grade 3 in all limbs, global hyperreflexia and frontal release signs were present. Brain MRI demonstrated multiple contrasting juxtacortical, hemispherical and bilateral nodular images, sometimes with diffusion restriction, as well as in the hypothalamic-chiasmatic region, medial thalamus, pontomesencephalic transition and right cerebellar hemisphere. There are also areas of discontinuous contracting delineating the ependymal surface of the lateral and III ventricles. It is associated with hypersignal on T2/FLAIR of the adjacent brain parenchyma, determining compression of regional cerebrospinal fluid (CSF). CSF examination showed cell count of 7,6/mm, protein 178 mg/dl, lactate 32 mg/dl, glucose 63 mg/dl and was negative infectious screening and oncotic citology. He had negative serologies. Incisional biopsy was performed in cervical lymph node, compatible with Diffuse Large B-cell Lymphomas (DLBCLs). Discussion: Isolated central nervous system (CNS) relapse involving the brain parenchyma is a rare complication of non-Hodgkin lymphoma. Secondary CNS involvement (SCNSL) affects 5% of patients with DLBCL. Cranial nerve signs, headache, mental status and personality changes were the most frequent manifestations. The neuroimaging characteristics may mimic other neurologic disorders. The immunocompetent patients with primary CNS lymphoma (PCNSL) presents uniformly enhancing mass lesions without necrosis. Intraparenchymal mass and perilesional edema are the most common neuroradiological findings suggesting a SCNSL. SCNSL predominantly presented with multiple lesions, in contrast to PCNSL, while infratentorial and brainstem involvement were rare in SCNSL. In our case, multiple nodulary lesions can mimic neurotuberculosis, nocardiosis and fungal infections. Final Comments: The suspicion of CSF lymphoma is a challenge in patients without previous diagnosis of aggressive lymphoma. Atypical MRI and CSF features make it difficult to confirm diagnosis. In this scenario, invasive strategies and investigating the primary site could be necessary.