Lymphomatosis Cerebri with Intramedullary Spinal Cord Involvement

Sato, Hiroyasu; Takahashi, Yusuke; Wada, Manabu; Shiono, Yousuke; Suzuki, Ikukatsu; Kohno, Kei; Kato, Yuichi; Kawanami, Toru; Sakurada, Kaori; Kayama, Takamasa; Kato, Takeshi

doi:10.2169/internalmedicine.52.0748

Cited by 13 publications

(13 citation statements)

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“…Among them, 10 cases reported 3 brain MRI examinations. In the second MRI, 24 cases showed progression (including the present case) ( 13 , 14 , 17 , 19 , 20 , 23 , 25 – 27 , 30 , 33 , 35 , 37 , 38 , 41 – 47 , 49 – 51 ) ( Figs. 2 and 3 ), 5 cases demonstrated reduction of lesions ( 28 , 32 , 36 , 37 , 39 ) and 2 cases showed no change from the initial scan ( 26 , 34 ).…”

Section: Resultsmentioning

confidence: 65%

“…In the second MRI, 24 cases showed progression (including the present case) ( 13 , 14 , 17 , 19 , 20 , 23 , 25 – 27 , 30 , 33 , 35 , 37 , 38 , 41 – 47 , 49 – 51 ) ( Figs. 2 and 3 ), 5 cases demonstrated reduction of lesions ( 28 , 32 , 36 , 37 , 39 ) and 2 cases showed no change from the initial scan ( 26 , 34 ). In the third MRI, when reported, 6 cases showed the enlargement of lesions ( 32 , 35 , 41 , 46 , 49 ) and 4 cases revealed the reduction of lesions ( 23 , 26 , 30 , 34 , 37 ).…”

Section: Resultsmentioning

confidence: 65%

“…2 and 3 ), 5 cases demonstrated reduction of lesions ( 28 , 32 , 36 , 37 , 39 ) and 2 cases showed no change from the initial scan ( 26 , 34 ). In the third MRI, when reported, 6 cases showed the enlargement of lesions ( 32 , 35 , 41 , 46 , 49 ) and 4 cases revealed the reduction of lesions ( 23 , 26 , 30 , 34 , 37 ). In 16 cases, lesion extension was described as spreading along the corticospinal tract down to the basal ganglia, thalamus, midbrain, pons, medulla oblongata and the left cerebellar hemisphere, even into the spinal cord.…”

Section: Resultsmentioning

confidence: 99%

“…In 16 cases, lesion extension was described as spreading along the corticospinal tract down to the basal ganglia, thalamus, midbrain, pons, medulla oblongata and the left cerebellar hemisphere, even into the spinal cord. Lesions extending through the corpus callosum were also observed ( 23 , 25 , 37 , 39 , 40 ).…”

Section: Resultsmentioning

confidence: 99%

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Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report

Rong

Feng

2018

Oncol Lett

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Lymphomatosis cerebri is a rare form of diffusely infiltrating primary central nervous system (CNS) lymphoma (PCNSL). The neuroradiological findings of lymphomatosis cerebri have not been adequately characterized, as the relevant literature consists only of case reports and small case series. The present study describes an unusual presentation of lymphomatosis cerebri in a 56-year-old immunocompetent woman who presented with diffusely infiltrating lesions with perivascular curvilinear enhancement on initial magnetic resonance imaging (MRI) and multiple nodules on the later follow-up computed tomography (CT) scan. A systematic review of the literature is also performed searching PubMed between January 1996 and December 2016 to collect all pertinent case reports and series written in the English language with pathologically confirmed lymphomatosis cerebri and diffuse infiltrative PCNSL without cohesive masses on initial MRI. A total of 45 cases were identified from 39 articles and the present case report. The patient ages ranged from 28 to 85 years (mean, 57.3 years). Only 3 patients (6.7%) were immunosuppressed (acquired immune deficiency syndrome patients). The most common clinical presentation was cognitive changes or dementia (46.7%). Cerebrospinal fluid analysis in all cases was non-specific. Diffuse and asymmetric abnormal T2-hyperintensity in deep and subcortical white matter was observed in all cases. Gray matter involvement (17.8%), spreading along the corticospinal tract (35.6%) and a slight mass effect (51.1%) also were observed. Contrast-enhanced patterns on MRI could be divided into three forms of non-enhancement (64.4%) and non-mass-like enhancement (35.6%) on initial MRI, as well as nodular or mass-like enhancement on the later follow-up MRI (15.6%). There were non-specific findings on magnetic resonance spectroscopy for 4 patients, on positron emission tomography/CT for 12 patients and on single-photon emission CT for 1 patient. Diagnosis was established by brain biopsy in 35 cases (77.8%) and autopsy in 9 cases (20%), involving B-cell lymphoma in 40 cases (88.9%) and T-cell lymphoma in 4 cases (8.9%). In conclusion, lymphomatosis cerebri, namely diffuse PCNSL or diffuse lymphoma of the CNS, is characterized by rapidly progressive dementia in the elderly, diffusely infiltrated CNS white matter along the corticospinal tract, possible involvement of the gray matter, a slight mass effect and varied contrast-enhancement patterns on MRI. Non-enhancement or non-mass-like enhancement on MRI may be a special form of diffuse PCNL during disease development and progression.

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Section: Resultsmentioning

confidence: 65%

Section: Resultsmentioning

confidence: 65%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report

Rong

Feng

2018

Oncol Lett

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“…Já convulsões são uma manifestação inicial rara, sendo descritas em menos de 5% dos pacientes. Eventualmente foram descritas ainda queixas menos frequentes, tais como cefaleia e vertigem 12 , hipotensão ortostática 10 , anorexia 7 e perda ponderal 9,11 .…”

Section: Manifestações Clínicasunclassified

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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Clinical and imaging features of lymphomatosis cerebri: analysis of 8 cases and systematic review of the literature

Fan,

Zhao,

Chen

et al. 2023

Clin Exp Med

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Lymphomatosis cerebri (LC) is a rare type of primary central nervous system lymphoma with diffuse, nonenhancing infiltrative lesions and is often misdiagnosed. Our study aimed to investigate the clinical characteristics and prognosis of LC through analyzing patients from the literature and our own center, so as to improve early diagnosis and treatment. PubMed, Web of Science and our hospital databases were reviewed, and information on demographic, clinical, pathological, cerebrospinal fluid (CSF), neuroimaging and treatment options was extracted. Univariate survival analysis was conducted by generating survival curves and comparing them using the log-rank test. Multivariate analysis was performed using the Cox proportional hazards regression model to identify the prognostic predictors. A total of 81 patients (median age: 58 years; interquartile range, IQR: 50–66.5 years), 45 males and 36 females, were included. The most common symptoms were cognitive impairment (65.4%) and gait impairment (50.6%). Imaging studies indicated that all 81 patients had supratentorial structure involvement, and 93.8% (76/81) had bilateral hemisphere involvement. There were 53.3% (32/60) patients with CSF pleocytosis and 65% (39/60) patients with increased CSF protein levels. The median time of diagnosis was 4.8 months (IQR: 2.3–6.9 months). Compared with 4 (95% CI: 1.78–6.22) months for all 81 patients, the median OS was 20 (95% CI: 8.24–31.76) months for those who had chemotherapy plus radiotherapy. Multivariate Cox analysis revealed that chemoradiotherapy (HR: 0.12; 95% CI: 0.02–0.68) and higher CSF glucose level (HR: 0.01; 95% CI: 0.00–0.26) were inversely associated with death. The diagnosis of LC should be alerted when neuroimaging with bilateral hemispheric involvement and CSF abnormality with pleocytosis and increased protein. Once the diagnosis is confirmed, the combination of chemotherapy and radiotherapy can be considered if the patient’s physical condition permits.Journal standard instruction requires an unstructured abstract. Kindly check and confirm.We have checked and confirmed that there is no problem.

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Lymphomatosis Cerebri with Intramedullary Spinal Cord Involvement

Cited by 13 publications

References 10 publications

Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report

Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report

Linfomatose Cerebral

Clinical and imaging features of lymphomatosis cerebri: analysis of 8 cases and systematic review of the literature

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