Primary non-Hodgkin's lymphomas of the CNS

Abstract

This paper reports on 10 patients (4 male, 6 female) with primary non-Hodgkin's lymphomas of the brain (CNS-NHL--mean age 46.8 years, mean postdiagnostic survival 10 months). Pathological CSF (cerebrospinal fluid) was found in all 8 patients examined (positive cytology in 7/8 cases). Solitary tumors, diffuse periventricular infiltration or diffuse cerebral infiltration were demonstrated in cerebral computer-assisted tomography (CAT). Angiographical findings were unspecific. The histologic subtypes were lymphoplasmacytoid immunocytoma (4), unclassified low grade (1), centroblastic (1), B-immunoblastic (1), T-immunoblastic (1), lymphoblastic convoluted T-cell type (1), unclassified high grade (1) NHL. Patients who had received radiotherapy (+/- surgery) in this group had a mean survival of 15.66 months (sigma = 7.63). In addition, an overview of 83 well-documented, cases of the literature tries to characterize main histological and topographical distributions, histology-, patient's age-, and therapy-related survival. Patients with primary CNS-NHL have a 5-year survival expectancy of 30% compared with 2.3% in secondary CNS-manifestations of systemic non-Hodgkin's lymphomas. In this report, the beneficial effect of radiotherapy (mean survival 30.3 months) compared to surgery or symptomatic treatment (3.6 or 3.3 months) could be confirmed. It is concluded that primary CNS-NHL frequently present with atypical neuropsychiatric syndromes; diagnosis should be established preferentially with CAT and CSF-examinations or stereotactic biopsies, whereas open surgery should be avoided. An approach to exact classification should be attempted, as survival is clearly related to histological subtypes.

Low‐grade primary central nervous system lymphoma in immunocompetent patients

“…8 in our study). Bogdahn et al (1986), Miller et al (1994) and Villegas et al (1997) reported a better survival with low-grade histology. Thus, local and symptom-oriented therapy may be the best approach for at least some patients with low-grade PCNSL, considering the risk of late neurotoxicity after intensive PCNSL treatment.…”

“…In the series of 10 patients reported by Bogdahn et al (1986), five patients (three males, two females, age 27-60 years) had a lowgrade histology: four had a lymphoplasmacytoid immunocytoma and one had an unclassified lymphoma. Remarkably, cerebellar localization and neoplastic cells in the CSF were present in each of the three patients.…”

“…The data on low-grade PCNSL is limited to a few case reports (Mikol et al, 1987;King et al, 1998;Braks et al, 2000) and pathological series (Jellinger et al, 1975;Bogdahn et al, 1986;Merkel & Hansmann, 1986;Simon et al, 1987;Kanav- 1990;Bergmann et al, 1993;Ferracini et al, 1993). Braks et al (2000) reported a 42-year-old woman with a 3-year history of headaches and vertigo and a 1-year history of seizures.…”

Management of Primary Central Nervous System Lymphoma

“…According to the Working Formulation 79% would have been classified as diffuse large-cell lymphoma in the REAL classification, whereas only 48.8% would have been interpreted as such in the updated Kiel Classification [28]. Perhaps this discrepancy explains in part the fact that some authors find a correlation with survival [20,28,51,52] while others do not [12,53]. Another classification awaited from the WHO may solve current problems [24].…”

T-Cell Lymphoma Showing a Non-Enhancing Diffuse White Matter Lesion with Marked Brain Atrophy.

“…Magnetic resonance (MR) images of MLshow two features similar to those of other brain tumors: expanding mass lesions and gadolinium (Gd)-enhancing lesions (3)(4)(5). Only a few cases with unusual MRIfindings are reported; these showeither Gd-enhancingdiffuse white matter lesions without mass effect (4,6) or non-Gd-enhancing "mass" lesions (7)(8)(9). Here we report a patient with T-cell lymphoma showing both non-Gd-enhancing and diffuse T2-high signal intensity lesions in the cerebral white matter.…”