title: Population-based experience on primary central nervous system lymphoma 2000-2012: the incidence is increasing.
abstract: BACKGROUND
  Primary central nervous system lymphomas (PCNSL) are rare lymphomas with a poor prognosis. Recently, an increased incidence has been reported. The present study is a population-based study of all patients with PCNSL in the Uppsala/Örebro region of middle Sweden.
  
  
  METHODS
  All patients diagnosed with a PCNSL at Uppsala University Hospital 2000-2012 were identified. Altogether, 96 patients (50 women and 46 men) were included. The median age at diagnosis was 66 years (17-95).
  
  
  RESULTS
  There was a statistically significant increase in age-standardized incidence during the study period, 30 patients were diagnosed in the first half and 66 in the second half of the period. No patient had an HIV-infection. Two patients had undergone kidney transplantation and were treated with immunosuppressive drugs. A high proportion of the patients, 29%, had a history of an autoimmune or inflammatory disease. The prognosis was poor with a median survival of only four months. In the 70 (73%) patients treated with curative intention the median survival was 12 months. Patients treated with high-dose methotrexate, radiotherapy and/or temozolomide appeared to have a better survival. There was no improvement in survival during the study period or after the introduction of rituximab. There also was no difference in any of the analyzed variables that could explain the increased incidence.
  
  
  CONCLUSIONS
  In this population-based study we could confirm the previously described increased incidence of PCNSL. The prognosis remains poor despite the inclusion of treatment with rituximab during the study period. A high proportion of the patients had a history of an autoimmune or inflammatory disease not previously described but there was no increase during the study period.
authors.affiliation: a Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology , Uppsala University , Sweden.
authors.forename: Gunilla
authors.initials: G
authors.lastname: Enblad
authors.affiliation: a Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology , Uppsala University , Sweden.
authors.forename: Gustaf
authors.initials: G
authors.lastname: Martinsson
authors.affiliation: b Department of Medical Sciences , Uppsala University , Sweden.
authors.forename: Eva
authors.initials: E
authors.lastname: Baecklund
authors.affiliation: c Department of Neurosciences , Uppsala University , Sweden.
authors.forename: Göran
authors.initials: G
authors.lastname: Hesselager
authors.affiliation: d Department of Immunology, Genetics and Pathology , Uppsala University , Sweden.
authors.forename: Christer
authors.initials: C
authors.lastname: Sundström
authors.affiliation: d Department of Immunology, Genetics and Pathology , Uppsala University , Sweden.
authors.forename: Rosie-Marie
authors.initials: RM
authors.lastname: Amini
authors.affiliation: a Department of Immunology, Genetics and Pathology, Experimental and Clinical Oncology , Uppsala University , Sweden.
authors.forename: Hans
authors.initials: H
authors.lastname: Hagberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D008223:Lymphoma / Q000188:drug therapy / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013548:Sweden / Q000453:epidemiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents