Affiliation:
1. Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
2. Department of Radiation Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
3. Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
4. Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Abstract
Background:Primary central nervous system-diffuse large B-cell lymphoma (PCNS-DLBCL) is a rare extra-nodal Non-Hodgkin lymphoma. There is relative paucity of literature on PCNSL from Indian subcontinent. We aimed to analyze the clinicopathological features of PCNSL and categorize them into germinal center B cell (GCB) and non-GCB subtypes to assess their prognostic significance in Indian context.Methods:All patients with histopathologically diagnosed PCNSLs at our center over a period of 6 years were recruited and classified into GCB and non-GCB using Han’s algorithm (immunohistochemistry for CD10, BCL6 and MUM1).In situhybridization (ISH) for Epstein-Barr virus (EBV)-encoded RNA was performed.Results:Eighty-six cases of PCNS-DLBCL were included with median age of 55 years. Majority of them were supratentorial in location (n= 62). All patients were immunocompetent. On immunohistochemical assessment, 69 (80.2%) were of NGCB subtype, 10 (11.6%) were of GCB subtype, and 7 (8.1%) were unclassified. Overall, MUM1, BCL-6, and CD10 expressions were seen in 69 (80.2%), 28 (32.6%), and 2 cases (2.3%), respectively. Four cases (4.6%) showed C-MYC expression. The median overall survival (OS) was 675 days. None of the factors (age, sex, location, immunomarkers, and GCB vs. NGCB phenotype) showed correlation with OS; however, BCL6 positive cases showed slight better OS (P> 0.05). All cases were negative for EBV-LMP1 on ISH.Conclusion:The majority of the CNS DLBCL belongs to non-GCB phenotype and uniformly carry poor prognosis, irrespective of their phenotype. Individual markers, such as BCL-6, MUM1, or CD10, are unable to predict outcome in PCNS-DLBCL.
Subject
Neurology (clinical),Surgery
Cited by
7 articles.
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