148P - Primary CNS lymphoma – Our experience with this rare disease
|Date||17 December 2016|
|Event||ESMO Asia 2016 Congress|
Central Nervous System Malignancies
|Citation||Annals of Oncology (2016) 27 (suppl_9): ix42-ix45. 10.1093/annonc/mdw578|
A. Pathak, A. Kapoor
Primary CNS lymphoma (PCNSL) is an an uncommon variant of extranodal non-Hodgkin lymphoma (NHL) arising exclusively in the CNS, that is, the brain parenchyma, spinal cord, eyes, cranial nerves, and/or meninges. It is a highly aggressive malignancy. This lymphoma represents 4% of intracranial neoplasms and 4%-6% of all extranodal lymphomas. The standard chemotherapy regimensfor treatment of systemic lymphoma (eg, CHOP), are either ineffective or too toxic. The definitive treatment of PCL is not known and there is a lot of institutional variation. Here we present our experience of managing 10 cases of PCNSL in immunocompetent patients, their initial presentation, diagnosis and their treatment.
We treated 10 patients in our tertiary care centre in the last year. The complete staging work up was done to exclude any other systemic lymphoma by whole body CECT scan, ophthalmologic evaluation (including slit-lamp examination), CSF studies, bone marrow biopsy, USG tests. Treatment given was on the basis of standard protocols.
We treated 10 patients in our tertiary care centre in the last year. We had two patients, one of 30 years and the other of 32 years, and the rest were older than 60 years. All the patients presented with headache and 60% (6/10) had instability of gait and 40% (4/10) had hemiparesis. Only 20% (2/10) had features of raised intracranial tension and one presented with vitreous haemorrhage. On MRI, there was a single lesion in 50% (5/10). The distribution of the solitary lesion was hemispheres 50% (5/10), periventricular 60% (6/10), thalamus/basal ganglia 40% (4/10), corpus callosum 20%, (2/10) and cerebellum (10%) (1/10). On histopathology 90% were diffuse large B cell lymphoma (9/10) and 10% (1/10) was T cell-rich B cell lymphoma (IHC – CD20+, LCA+, CD3+).
Though there has been no clear evidence as to the best management protocol, we observed that the addition of second agent was entirely dependent on the clinical status of the patient. However with expansion of molecular biology and improvement in diagnostic sensitivity and specificity in coming years we can perhaps reduce the dilemma of dose intensive versus de-escalation therapy in management of PCNSL.
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All authors have declared no conflicts of interest.