Open Access

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

  • Authors:
    • Long Li
    • Jia‑Hui Rong
    • Jie Feng
  • View Affiliations

  • Published online on: May 30, 2018     https://doi.org/10.3892/ol.2018.8839
  • Pages: 1463-1474
  • Copyright: © Li et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )
Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )


Abstract

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.
View Figures
View References

Related Articles

Journal Cover

August-2018
Volume 16 Issue 2

Print ISSN: 1792-1074
Online ISSN:1792-1082

Sign up for eToc alerts

Recommend to Library

Copy and paste a formatted citation
x
Spandidos Publications style
Li L, Rong JH and Feng J: Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report. Oncol Lett 16: 1463-1474, 2018
APA
Li, L., Rong, J., & Feng, J. (2018). Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report. Oncology Letters, 16, 1463-1474. https://doi.org/10.3892/ol.2018.8839
MLA
Li, L., Rong, J., Feng, J."Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report". Oncology Letters 16.2 (2018): 1463-1474.
Chicago
Li, L., Rong, J., Feng, J."Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report". Oncology Letters 16, no. 2 (2018): 1463-1474. https://doi.org/10.3892/ol.2018.8839