Non-Hodgkin Lymphomas of the Central Nervous System: Clinico-Pathologic and Immunohistochemical Study of 147 Cases

Abstract

We report on data gathered from five European centres regarding 147 primary non-Hodgkin Lymphomas (NHLs) of the Central Nervous System (CNS) in HIV-negative patients. The results lead us to make the following considerations: i) there has been a significant and progressive increase in the frequency of observation of this pathology during the course of the last two decades; ii) the pathology lacks specific characteristic symptoms; iii) the radiological profile, as observed by CAT and/or MNR, most frequently corresponds to an isodense or slightly hyperdense lesion which has clear margins and is capable of assuming the contrast medium homogeneously; iv) the tumour most often has a single supratentorial localisation; v) high grade B-cell lymphomas account for 66% of the observations, low grade B-cell varieties being relatively rare and cases of T-cell derivation exceptional (6/147); vi) immunohistochemistry allows the differential diagnoses with respect to primitive or secondary non-lymphoid tumours, and provides confirmation of the histogenetic assessment made on morphological grounds; vii) the course of the disease is not significantly influenced by the histotype, the phenotype, the number of lesions present or the chemotherapy regimen, but rather by the employment of combined surgery and radio- or radiochemotherapy. This study represents the largest series of CNS NHLs so far reported, and as such, provides precise clinico-pathological indications which were only partially obtainable from the relatively small previously published series. Some concluding remarks are made as to the genesis of CNS NHLs, along with some practical suggestions for reaching a better understanding of their complex biology.