%0 Journal Article
%T Treatment of Primary Central Nervous System Lymphoma: From Chemotherapy to Small Molecules
%A Christian Grommes
%A Joe S. Mendez
%J About the Ed Book | ASCO Educational Book
%D 2018
%R https://doi.org/10.1200/EDBK_200829
%X PCNSL is a highly aggressive non-Hodgkin lymphoma confined to the central nervous system (CNS), including the brain, spine, CSF, and eyes. Unlike other primary brain tumors, it often has a favorable response to both chemotherapy and radiation therapy; however, compared with lymphomas outside the CNS, survival is usually inferior. Moreover, the prognosis for PCNSL that fails to respond to first-line therapy remains poor. Even though new chemotherapy-based therapeutic approaches have improved survival, the management of this disease still poses a challenge in neuro-oncology. EPIDEMIOLOGY Section: ChooseTop of pageAbstractEPIDEMIOLOGY <<CLINICAL PRESENTATIONDIAGNOSIS AND STAGINGPROGNOSISEVOLUTION OF STANDARD THE...NOVEL INSIGHTSSMALL MOLECULES APPLIED I...FUTURE DIRECTIONSCONCLUSIONACKNOWLEDGMENTReferences PCNSL can develop in patients who are immunosuppressed (HIV/AIDS, organ transplantation, immunosuppressive agents) or immunocompetent. In this review, we will focus on the latter. PCNSL in the immunocompetent patient is rare and represents only 4% of all intracranial neoplasms and 4% to 6% of all extranodal lymphomas.1 Approximately 1,500 new patients are diagnosed each year in the United States. In recent years, an increasing incidence has been recognized in patients older than age 60, in whom the annual incidence rate is 0.5 per 100,000.2 The overall incidence rate of PCNSL has remained stable since the mid-1980s,3 other than a sharp spike in PCNSL incidence in the early 1990s that most likely was due to the HIV epidemic. The overall increase in PCNSL seen in the 1980s is a reflection of a growing elderly population with a longer life expectancy. The highest incidence rates are observed in patients age 60 and older, and the highest rates are in those age 70 to 79 (4.3 per 100,000 per year).3 CLINICAL PRESENTATION Section: ChooseTop of pageAbstractEPIDEMIOLOGYCLINICAL PRESENTATION <<DIAGNOSIS AND STAGINGPROGNOSISEVOLUTION OF STANDARD THE...NOVEL INSIGHTSSMALL MOLECULES APPLIED I...FUTURE DIRECTIONSCONCLUSIONACKNOWLEDGMENTReferences Diagnosis of PCNSL requires a high level of suspicion, because clinical presentations vary according to the involved compartments. Focal neurologic deficits, which result from involvement of the parenchyma or leptomeninges, lead to prompt imaging but are only seen in 70% of patients.4 Up to 43% of individuals have behavioral or neuropsychiatric changes that are nonspecific, which can lead to a delay in medical evaluation. Signs of elevated intracranial pressure, such as headache, nausea, and vomiting, are also common (33%).
%U http://ascopubs.org/doi/full/10.1200/EDBK_200829