title: [Special hematological diagnostics and therapy options for ocular lymphoma taking CNS involvement into account].
abstract: BACKGROUND
  Intraocular lymphomas are very rare and occur as either vitreoretinal or uveal tumors. Management in the clinical routine is highly variable and controversial.
  
  
  OBJECTIVE
  To present the most important aspects of the diagnostics and therapy from the perspective of hematological oncologists and formulate management recommendations.
  
  
  METHODS
  The English language literature was reviewed and the most important data were analyzed for presentation.
  
  
  RESULTS
  In patients with vitreoretinal lymphoma evaluation for central nervous system (CNS) involvement should be performed due to its strong association with primary CNS lymphoma (PCNSL). The prognosis is relatively poor, particularly when the CNS is involved. Optimal therapy has not yet been established. For isolated vitreoretinal manifestations local therapy, such as intraocular methotrexate (MTX) or rituximab or radiation is recommended; however, there is a very high frequency of CNS relapse. Systemic high-dose MTX-based chemotherapy analogous to PCNSL treatment is an alternative option and is the treatment of choice in patients with simultaneous CNS and vitreoretinal lymphoma. Primary uveal lymphoma is usually an indolent lymphoma and treated by local therapy, whereas secondary uveal lymphoma predominantly occurs in aggressive systemic (non-CNS) lymphoma and is treated by systemic chemotherapy.
  
  
  CONCLUSIONS
  Data on intraocular lymphoma are derived from small, usually retrospective and very heterogeneous studies with a relatively short follow-up. To gain more knowledge on this rare disease, inclusion of patients in the prospective registry, currently in progress in Germany, is desirable.
authors.affiliation: Klinik für Hämatologie, Onkologie und Tumorimmunologie, Charité - Universitätsmedizin Berlin, Hindenburgdamm 30, 12200, Berlin, Deutschland, agnieszka.korfel@charite.de.
authors.forename: A
authors.initials: A
authors.lastname: Korfel
authors.forename: U
authors.initials: U
authors.lastname: Schlegel
authors.forename: R
authors.initials: R
authors.lastname: Schroers
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D003937:Diagnosis, Differential; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis* / Q000628:therapy*; D009361:Neoplasm Invasiveness
chemical_list: D000970:Antineoplastic Agents