• Mariana Nassif Kerbauy, Denise da Cunha Pasqualin, Jerusa Smid, Rogerio Iquizli, Kerbauy Lucila Nassif LN Department of Hematology, Hospital Israelita Albert Einstein., Ricardo Nitrini, Guilherme Carvalhal Ribas, Luciano Neder, and Nelson Hamerschlak.
• Department of Hematology, Hospital Israelita Albert Einstein.
• Medicine (Baltimore). 2019 Feb 1; 98 (6): e14367.

RationaleLymphomatosis cerebri is a rare form of PCNSL, characterized by diffuse infiltration of lymphoma cells in cerebral parenchyma, without mass-formation and mild or no contrast enhancement on magnetic resonance (MR) imaging. There are less than 50 cases described in the literature under the term Lymphomatosis cerebri.Patient ConcernsA 74-year-old man presented to our service with progressive dementia for 12 months and accelerated cognitive decline within the last two months. Brain magnetic resonance imaging showed areas of hyperintensity involving predominantly the white matter of frontal lobes and knee of the corpus callosum, along with areas of blood-brain barrier disruption and areas of restricted diffusion. Stereotaxy brain surgery was indicated into contrasting areas and histologically there was heterogeneous foci of discreet infiltration of rare medium-large lymphoid cells intermingled with inflammatory cells and these atypical lymphoid cells were placed on breakdown neuropil and did not form tumor mass or sheets of cells, but occasionally displayed perivascular distribution. Immunohistochemically, these atypical lymphoid cells expressed CD20, Bcl2, Bcl6 and, heterogeneously, IRF4/MUM1.DiagnosisThe diagnosis of a primary CNS diffuse large B-cell lymphoma manifested as lymphomatosis cerebri was performed.InterventionsThe treatment of choice was: temozolomide 100 mg/m (D1 to D5), methotrexate 3 g/m (D1, D10, and D20) and rituximab 375 mg/m.OutcomesThe patient evolved with progressive neurological deterioration, regardless of the improvement on neuroimaging.LessonsWe described the diagnostic dilemma we faced with an elderly man with rapid cognitive impairment and a myriad of differential diagnoses, diagnosed with primary CNS diffuse large B-cell lymphoma with a lymphomatosis cerebri-like pattern.

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