title: [Diagnostics and treatment of primary vitreoretinal lymphoma].
abstract: BACKGROUND
  Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, mostly a diffuse large B-cell lymphoma. The PVRL, previously called primary intraocular lymphoma (PIOL), is a subset of primary central nervous system lymphoma (PCNSL).
  
  
  METHODS
  The diagnosis of PVRL is often difficult as it often mimics chronic intermediate or posterior uveitis; therefore, PVRL requires various procedures for the diagnostics, e.g. immunohistochemistry, cytology, pathology, molecular pathology and cytokine analysis (interleukin 10) after surgically obtaining ocular specimens.
  
  
  METHODS
  Treatment forms that are effective for systemic lymphomas have not been reliably successful for PVRL and PCNSL. Current management of PVRL consists of chemotherapy, such as methotrexate or rituximab, possibly combined with external beam radiation whereby both chemotherapeutic agents are administered systemically as well as intravitreally. Intravitreal treatment alone is recommended solely in the case of monocular PVRL, which is highly controversial. A PVRL usually responds well to initial treatment; however, relapse rates and CNS involvement are high, resulting in a poor prognosis and limited survival.
authors.affiliation: Augenklinik, Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12203, Berlin, Deutschland, nicole.stuebiger@charite.de.
authors.forename: N
authors.initials: N
authors.lastname: Stübiger
authors.forename: V
authors.initials: V
authors.lastname: Kakkassery
authors.forename: E
authors.initials: E
authors.lastname: Gundlach
authors.forename: S
authors.initials: S
authors.lastname: Winterhalter
authors.forename: U
authors.initials: U
authors.lastname: Pleyer
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D059248:Chemoradiotherapy / Q000379:methods*; D003937:Diagnosis, Differential; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D019572:Retinal Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents