Diffuse large B-cell lymphoma of the central nervous system presenting as ""lymphomatosis cerebri"" and dementia in elderly man: Case report and review of the literature

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art_KERBAUY_Diffuse_large_Bcell_lymphoma_of_the_central_nervous_2019.PDF (1.05 MB)
Citações na Scopus
14
Tipo de produção
article
Data de publicação
2019
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
LIPPINCOTT WILLIAMS & WILKINS
Autores
KERBAUY, Mariana Nassif
PASQUALIN, Denise da Cunha
IQUIZLI, Rogerio
KERBAUY, Lucila Nassif
RIBAS, Guilherme Carvalhal
NEDER, Luciano
HAMERSCHLAK, Nelson
Citação
MEDICINE, v.98, n.6, article ID e14367, 9p, 2019
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Rationale:Lymphomatosis 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 concerns:A 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.Diagnosis:The diagnosis of a primary CNS diffuse large B-cell lymphoma manifested as lymphomatosis cerebri was performed.Interventions:The treatment of choice was: temozolomide 100mg/m(2) (D1 to D5), methotrexate 3g/m(2) (D1, D10, and D20) and rituximab 375mg/m(2).Outcomes:The patient evolved with progressive neurological deterioration, regardless of the improvement on neuroimaging.Lessons:We 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.
Palavras-chave
dementia, diffuse lymphoma, large B-Cell, lymphoma, nervous system neoplasms
URI
https://observatorio.fm.usp.br/handle/OPI/31737
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MNE
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/15
Artigos e Materiais de Revistas Científicas - ODS/03