Diagnosis, prognosis and treatment of primary central nervous system lymphoma in the elderly population (Review)

Abstract

Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma that is unique and different from systemic diffuse large B-cell lymphomas. The median age at diagnosis of PCNSL is 65 years and its incidence is rising rapidly in the elderly population. A total of ≥20% of all patients with PCNSL are ≥80 years old. Notably, age has been identified as an independent poor prognostic factor for PCNSL. Elderly patients have an inferior prognosis to that of younger patients and are more severely affected by iatrogenic toxicity; therefore, elderly patients represent a unique and vulnerable treatment subgroup. The present review summarized the available literature to provide an improved understanding of the epidemiology, clinical characteristics, diagnosis, prognosis and management of PCNSL in the elderly population. Notably, the incidence of PCNSL in immunocompetent elderly patients, predominantly in men, is increasing. For the diagnosis of CNSL, imaging-guided stereotactic biopsy is considered the gold standard. When stereotactic biopsy is not possible or conclusive, certain biomarkers have been described that can help establish a diagnosis. PCNSL has a very poor prognosis in the elderly, even though several prognostic scoring systems exist and several prognostic markers have been reported in patients with PCNSL. Furthermore, the treatment of elderly patients remains challenging; it is unlikely that a novel agent could be used as a curative monotherapy; however, a combination of novel agents with polychemotherapy or its combination with other novel drugs may have therapeutic potential. Contents1. Epidemiology 2. Clinical manifestation 3. Brain imaging

Lymphoma and the Microenvironmental Cross-Talk between Sex Hormone Receptors and Epstein-Barr Virus in Predicting Lymphoma Clinical Status

“…Even though numerous prognostic grading systems exist, and several prognostic markers have been discovered in PCNSL patients, the elderly have a very dismal prognosis. Furthermore, treating older individuals remains difficult; while a novel agent is unlikely to be utilized as a curative monotherapy, a combination of novel medicines with polychemotherapy or with other innovative therapies may have therapeutic potential [18].…”

Primary Central Nervous System Lymphoma With Systemic Recurrence

“…Primary central nervous system lymphomas (PCNSLs) are extranodal non-Hodgkin’s lymphomas confined to the brain, spinal cord, eyes, or leptomeninges [ 1 ]. They are rare and account for 2-4% of central nervous system tumors among immunocompetent patients and less than 1% of non-Hodgkin lymphomas [ 1 , 2 ]. The vast majority of PCNSLs are diffuse large B-cell lymphomas, however, rarely they could be of other histologic types [ 1 , 2 ].…”

“…They are rare and account for 2-4% of central nervous system tumors among immunocompetent patients and less than 1% of non-Hodgkin lymphomas [ 1 , 2 ]. The vast majority of PCNSLs are diffuse large B-cell lymphomas, however, rarely they could be of other histologic types [ 1 , 2 ]. The median age of patients diagnosed with PCNSLs is 66 years, with more than 20% of patients older than 80 years at diagnosis [ 1 , 2 ].…”

“…The vast majority of PCNSLs are diffuse large B-cell lymphomas, however, rarely they could be of other histologic types [ 1 , 2 ]. The median age of patients diagnosed with PCNSLs is 66 years, with more than 20% of patients older than 80 years at diagnosis [ 1 , 2 ]. The pathogenesis of the disease is incompletely understood but has been postulated to be due to either malignant transformation within the central nervous system or a systemic lymphoma that migrates to and preferentially grows within the central nervous system [ 3 ].…”

“…Though improved with the introduction of high-dose methotrexate-containing regimens, survival among patients with PCNSL lags behind that of systemic lymphomas [ 1 , 2 ]. The five-year survival rate is low in part because 35-60% of patients with PCNSL who achieve a complete response will relapse within five years [ 4 , 5 ].…”