Critical revision of the role of surgery for treatment of primary brain b cell lymphoma in an immunocompetent patient: a case report and literature review

Abstract

Primary Central Nervous System Lymphoma (PCNSL) consists in diffuse large B cell non-Hodgkin lymphoma usually confining to the CNS. PCNSL is well known to be a form of extra nodal, highgrade non-Hodgkin B-cell neoplasm. It can originate in the brain, leptomeninges, spinal cord, or eyes; usually it remains confined to the CNS, rarely it spreads outside the nervous system. It was previously considered as a rare tumor accounting for less than 2% of cerebral neoplasms, PCNSL is now being found with increasing frequency in immunocompetent patients. Although the cells of origin are lymphocytes (usually large cell or immunoblastic type), PCNSL should be considered a brain tumor, because its therapeutic challenges resemble those of other brain tumors. In particular, drug delivery is impaired by the blood-brain barrier, and cerebral toxicity limits the use of available treatment strategies. Most PCNSLs (about 90%) are diffuse large B-cell lymphomas (DLBCLs); the remaining 10% are poorly characterized low-grade lymphomas, Burkitt lymphomas, and T-cell lymphomas.1 Primary symptoms may be the sum of local mass effect due to raised intracranial pressure, or ocular involvement, or from focal deposits on cranial or spinal nerve roots. PCNSL incidence has risen steadily since the end of the 20th century. Incidence in immunocompetent patients is approximately 51 cases per 10,000,000 per year. Among immunocompetent patients with PCNSL, males outnumber females by approximately 2:1 The median age of immunocompetent patients with PCNSL is 55 years.2