Kanwardeep Singh Kwatra1, Preethi Anni Mercy Paul2, Saurabh Donald3, Roma Isaacs4, Pamela Alice Kingsley5
BACKGROUND
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma (NHL) confined to the brain, spinal cord and/or eye, occurring in immunocompetent individuals. Histologically, they are diffuse large B-cell lymphomas. Over the last few decades there has been a gradual increase in their incidence.
AIM
To study the clinical, histopathological and immunohistochemical profile of primary central nervous system lymphoma.
SETTING AND DESIGN
Retrospective audit of seven cases of PCNSL diagnosed over a period of five years in a tertiary referral hospital of North India.
MATERIAL AND METHODS
The clinical, radiological and laboratory findings were retrieved from the hospital records. Histopathology slides were reviewed, studied in detail and a panel of immunohistochemical markers comprising of CD3, CD5, CD20, CD10, BCL6, BCL2, MUM1, CD30, EBV (LMP1), Ki-67 and p53 was done on all cases.
RESULTS
The male to female ratio was 3:4 with a median age of 60 years. The most common form of presentation was neurological deficits and altered sensorium. Imaging showed contrast enhancing, single or multiple, deep seated lesions within the cerebral hemispheres. Histologically, all were high-grade diffuse large B-cell lymphomas showing typical angiocentricity and a median Ki-67 proliferative index of 80%. Based on immunohistochemistry (Hans classifier) three cases had germinal centre B-cell (GCB) and four had non-germinal centre B-cell (non-GCB) phenotype. p53 was expressed in all cases with strong expression in four of them. Four patients died before treatment could be initiated, one received palliative chemo-radiotherapy and two did not follow up after diagnosis.
CONCLUSIONS
Primary CNS lymphomas are high-grade diffuse large B-cell lymphomas which show high Ki-67 proliferative indices and frequent overexpression of p53. Irrespective of histological subtype, GCB or non-GCB, outcome is uniformly poor. Early and prompt diagnosis is essential to improve the dismal prognosis of these patients. Developing new targeted therapies is the need of the hour.
