Conference Proceeding

Primary B cell CNS lymphoma in animmunocompetent adolescent boy

Dr. B. Hemaswetha,
Sri Ramachandra Medical College & Research institute, Chennai, India

Dr. B. Hemaswetha is pursuing MD Pediatrics (II Year) at Sri Ramachandra Medical College and Research Institute, Chennai, India. Dedicated and passionate towards her chosen profession. Completed MBBS (UG) from the same Institute. Did schooling from Abu Dhabi Indian School. Won the prestigious Sheikh Hamdan Bin Rashid Al-Maktoumaward for the Most Distinguished Student in 2006. In SRMC&RI, Attended medical camps, Presented interesting topics in Clinical meets, participated in CME meets. Presented Poster at Chettinad PEDICON 2016. A certified ACLS, BLS, Pediatric Advanced Life Support (PALS) Provider and an Advanced NRP provider.

Introduction: Primary central nervous system non-hodgkinlymphoma (PCNSL) is a rare malignant tumor limited to the cranio-spinal axis, accounting for fewer than 5% of all cases of primary intracranial neoplasms. PCNSL typically exhibited in immunodeficient individuals and it is rare amongimmunocompetents. PCNSL is a highly radiosensitive and chemo sensitive infiltrative tumor, so surgery is restricted only to diagnostic Biographypsy. In this case report we intend to present about an adolescent immuno competent boy with PCNSL who attained remission with chemotherapy alone.
Case report: 17 year old, developmentally normal boy presented with signs and symptoms of raised intracranial pressure. Neuroimaging revealed a mass of 5x4.2x4.6 cm with extensive perilesional edema in left gangliocapsular, corona radiata and temporal lobe.Histopathological examination and immunohistochemistry of the lesion was suggestive of B cell Lymphoma. CECT (thorax and abdomen), CSF analysis and bone marrow Biographypsy ruled out metastatic disease. Retroviral serology was negative. He was treated as per LMB 96 protocol for CNS positive B cell NHL with high dose Methotrexate (5 gms/m2). MRI brain after 3 cycles of chemotherapy revealed significant reduction in the size of the lesion (1x0.5x1cm) and Whole body PET CT after 4 cycles was suggestive of disease in remission.
Discussion: A significant increase in the incidence of PCNSL was seen among elderly immunocompetent individuals (> 60 years) in the recent decade. There is no consensus regarding the optimal management of patients with PCNSL. The prognosis of patients with PCNSL has improved during the past decade with the introduction of high-dose methotrexate with whole brain radiotherapy. However, despite recent progress, results following treatment are durable only in few patients, and therapy can be associated with late neurotoxicity. PCNSL is an uncommon tumor, and no phase III trial has been completed so far, leaving many questions about its optimum first-line and salvage treatments unanswered.
Conclusion: Because long-term survivors are at a higher risk for developing severe delayed cognitive dysfunctions, future treatment should improve efficacy while limiting the risk for neurotoxicity. This excellent response favor’s the chemotherapy as the sole treatment of PCNSL but larger study population is needed to optimize the treatment guidelines.

Published: 11 May 2017