TY - JOUR
T1 - Diffuse large B-cell lymphoma of the central nervous system presenting as "lymphomatosis cerebri" and dementia in elderly man
T2 - Case report and review of the literature
AU - Kerbauy, Mariana Nassif
AU - Pasqualin, Denise Da Cunha
AU - Smid, Jerusa
AU - Iquizli, Rogerio
AU - Kerbauy, Lucila Nassif
AU - Nitrini, Ricardo
AU - Ribas, Guilherme Carvalhal
AU - Neder, Luciano
AU - Hamerschlak, Nelson
N1 - Publisher Copyright:
© 2019 the Author(s). Published by Wolters Kluwer Health, Inc..
PY - 2019/2/1
Y1 - 2019/2/1
N2 - 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/m2 (D1 to D5), methotrexate 3g/m2 (D1, D10, and D20) and rituximab 375mg/m2.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.
AB - 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/m2 (D1 to D5), methotrexate 3g/m2 (D1, D10, and D20) and rituximab 375mg/m2.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.
KW - dementia
KW - diffuse lymphoma
KW - large B-Cell
KW - lymphoma
KW - nervous system neoplasms
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U2 - 10.1097/MD.0000000000014367
DO - 10.1097/MD.0000000000014367
M3 - Review article
C2 - 30732171
AN - SCOPUS:85061292019
SN - 0025-7974
VL - 98
JO - Medicine (United States)
JF - Medicine (United States)
IS - 6
M1 - e14367
ER -