Lymphomatosis cerebri: a rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature

Izquierdo, Cristina; Velasco, Roser; Vidal, Noemí; Sánchez, Juan José; Besora, Sarah; Graus, Francesc; Bruna, Jordi

doi:10.1093/neuonc/nov197

Cited by 44 publications

(81 citation statements)

References 45 publications

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“…However, other reports have demonstrated that more than 50% of 42 patients had lymphomatosis identified in the basal ganglia with concurrent supratentorial and infratentorial lesions as well as bilateral hemispheric lesions 7. They are not associated with a mass effect.…”

Section: Discussionmentioning

confidence: 89%

“…The 5-year survival after combined chemotherapy and radiation therapy for PCNSL was reported in one report to be 32% 18. However, in a recent series of LC cases, 47.2% of patients did not survive past 3 months after the onset of symptoms 7. They found that a high Karnofsky score ≥70 and treatment with methotrexate was associated with improved survival and that T-cell LC is an independent poor prognostic factor.…”

Section: Discussionmentioning

confidence: 95%

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Lymphomatosis cerebri: diagnostic challenges and review of the literature

Lee

Berrios²,

Ionete

et al. 2016

BMJ Case Reports

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Lymphomatosis cerebri (LC) is a rare variant of a primary central nervous system non-Hodgkin's lymphoma (PCNSL) characterised by diffuse infiltration of tumour cells throughout the brain parenchyma. We present a 68-year-old immunocompetent woman with headaches, dizziness, blurred vision, localised right leg weakness and rapidly progressive dementia. A brain MRI demonstrated diffuse T2 hyperintense white matter lesions that did not enhance with contrast. The clinical differential diagnosis of these lesions included metastatic disease, infectious or inflammatory process such as sarcoidosis, lymphoma, demyelinating disease and less likely vascular aetiology, such as vasculitis or ischaemic stroke. A right frontal stereotactic brain biopsy was non-diagnostic. The patient eventually died from aspiration pneumonia following a pneumonectomy for a primary lung adenocarcinoma. The diagnosis of LC was established on postmortem examination of the brain.

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 95%

Lymphomatosis cerebri: diagnostic challenges and review of the literature

Lee

Berrios²,

Ionete

et al. 2016

BMJ Case Reports

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“…Até o momento, na literatura médica há apenas 27 artigos que se referem diretamente à linfomatose cerebral, descrevendo um total de 44 indivíduos com esta doença 5,6,8 .…”

Section: Bakshi Et Al 1 Descreveram Dois Casos De Indivíduos Com Demunclassified

“…A maior parte dos indivíduos são europeus (43,2%), seguidos pelos asiáticos (34,1%) e, por último, norte-americanos e canadenses (22,7%). Apenas raramente os indivíduos são imunossuprimidos (6,8%) 5,6,8 . Nossa paciente era do gênero feminino, dentro da faixa etária descrita e imunocompetente.…”

Section: Aspectos Demográficosunclassified

“…Por ocasião do diagnóstico, metade dos pacientes se encontram com KPS 70. Conforme a literatura, achados frequentes são o declínio cognitivo (59,1%), os distúrbios de marcha (56,8%) e alterações do comportamento (50%) 5,6,8 . No caso descrito, a procura do atendimento ocorreu aos 30 dias de evolução e o valor de seu KPS encontravase inferior à média descrita na literatura.…”

Section: Manifestações Clínicasunclassified

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Linfomatose Cerebral

Santos¹,

Vinholi²,

Silva³

et al. 2018

jbnc

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RESUMOA linfomatose cerebral (LC) é considerada uma rara variante do linfoma primário do sistema nervoso central (SNC). Descrevemos o caso de uma paciente com demência rapidamente progressiva secundária a extensa lesão em substância branca e núcleos da base bilateralmente, com evidente impregnação pelo contraste paramagnético. Dada a dificuldade diagnóstica, a paciente foi submetida a biópsia que revelou achados compatíveis com LC. Apesar do tratamento com metilprednisolona, ciclofosfamida e azatioprina, em 5 meses a paciente evoluiu para óbito. Devido à evolução subaguda e neuroimagem atípica, esta possibilidade diagnóstica pode não ser aventada e, com isso, não realizar a biopsia cerebral. O tratamento se baseia em altas doses de quimioterapia com metotrexato, sem indicação de ressecção tumoral. Contudo, sabe-se que é uma neoplasia de baixa resposta ao tratamento e com prognóstico reservado. Palavras-chave: Linfomatose cerebral; Linfoma; Demência rapidamente progressiva.ABSTRACT Lymphomatosis cerebri (LC) is considered a rare variant of primary lymphoma of the central nervous system (CNS). A patient with rapidly progressive dementia secondary to bilateral extensive injury in white matter and basal ganglia is described. Impregnation of paramagnetic contrast was intense. Given the diagnosis difficulty, the patient underwent biopsy revealing findings compatible with LC. Despite the treatment using methylprednisolone, cyclophosphamide, and azathioprine, after 5 months the patient evolved to death. Due to the subacute development, and atypical neuroimaging results, this diagnosis possibility would not be considered, and the brain biopsy not performed. Treatment is based on high-dose chemotherapy with methotrexate, and no indication of tumor resection. However, it is known that this neoplasm has low response to the treatment and poor prognosis.

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