Diffuse leukoencephalopathy due to lymphomatosis cerebri: a clinicopathological report

Imperiale, Daniele; Taraglio, Stefano; Atzori, Cristiana; Testi, Roberto

doi:10.1007/s10072-014-1974-3

Cited by 8 publications

(12 citation statements)

References 5 publications

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“…The lesions most commonly affected the deep and periventricular white matter, and predominantly involved white matter regions of the frontal, parietal, occipital and temporal lobes. Subcortical white matter hyperintensity and U-fiber involvement were frequently observed (24 cases, 53.3%; including the present case) ( 13 – 15 , 17 – 19 , 22 , 25 , 26 , 29 , 32 , 34 , 36 , 38 , 40 , 42 , 43 , 45 , 48 – 50 ). The lesions involved the corpus callosum in 19 cases (42.2%), the basal ganglia in 15 cases (33.3%), the thalamus in 12 cases (26.7%), the midbrain in 21 cases (46.7%), the pons in 20 cases (44.5%), the medulla in 14 cases (31.1%), the cerebellum in 12 cases (26.6%) and the spinal cord in 5 cases (11.1%) ( Table II ).…”

Section: Resultsmentioning

confidence: 50%

“…18 F-FDG PET/CT findings were mentioned in 12 patients, including 4 cases that revealed high uptake (including the present case) ( 25 , 47 , 50 ) ( Fig. 4 ), 3 cases with decreased uptake ( 31 , 36 , 47 ) and 5 cases with normal metabolism ( 23 , 26 , 29 , 34 , 43 ).…”

Section: Resultsmentioning

confidence: 71%

“…4 ). In 8 cases with a non-enhancing pattern on initial MRI, follow-up contrast enhancement MRI revealed patchy enhancement in 5 cases ( 30 , 35 , 42 , 43 , 47 ), single nodular enhancement in 1 case ( 37 ) and mass-like enhancement in 2 cases ( 35 , 50 ). In 1 case that presented as a slight enhancement on initial MRI, mass-like enhancement was subsequently found at 4 months post-admission ( 35 ).…”

Section: Resultsmentioning

confidence: 97%

“…The lesions involved the corpus callosum in 19 cases (42.2%), the basal ganglia in 15 cases (33.3%), the thalamus in 12 cases (26.7%), the midbrain in 21 cases (46.7%), the pons in 20 cases (44.5%), the medulla in 14 cases (31.1%), the cerebellum in 12 cases (26.6%) and the spinal cord in 5 cases (11.1%) ( Table II ). Lesions were also observed in the cerebral ( 20 , 28 , 41 , 43 , 50 ) and cerebellar ( 35 , 41 ) cortices, and the cerebellar dentate nuclei ( 30 ).…”

Section: Resultsmentioning

confidence: 99%

“…Contrast enhancement patterns on MRI could be divided into three forms: i) Non-enhancing (64.4%)( 13 – 24 , 26 – 28 , 30 , 32 , 33 , 37 , 38 , 42 , 43 , 45 , 46 , 48 – 51 ) on initial MRI; ii) non-mass-like enhancement (35.6%) on initial MRI; and iii) nodular or mass-like enhancement on follow-up MRI (15.6%). The non-mass-like enhancements on initial MRI were described as subtle (subtle, faint, slight, little and minimal) in 7 cases ( 34 – 36 , 39 , 40 , 44 , 47 ), patchy in 5 cases ( 22 , 29 , 32 , 48 ) and curvilinear in 4 cases (including the present case) ( 25 , 37 , 41 ) ( Fig.…”

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: 50%

Section: Resultsmentioning

confidence: 71%

Section: Resultsmentioning

confidence: 97%

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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Lymphomatosis cerebri: a rare diffuse leukoencephalopathy you should never miss

2022

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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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Diffuse leukoencephalopathy due to lymphomatosis cerebri: a clinicopathological report

Cited by 8 publications

References 5 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

Lymphomatosis cerebri: a rare diffuse leukoencephalopathy you should never miss

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

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