title: Intraocular lymphoma 2000-2005: results of a retrospective multicentre trial.
abstract: BACKGROUND
  The prognosis of intraocular lymphoma (IOL) is poor, and the optimal treatment has yet to be defined. This study assesses the clinical characteristics and outcome of patients with IOL diagnosed and treated in the new millennium.
  
  
  METHODS
  Patient data in this retrospective multicentre study were compiled by standardised questionnaires sent to seven university ophthalmology departments. All cases diagnosed with primary and secondary IOL in the past 5 years not associated with HIV infection were included.
  
  
  RESULTS
  Twenty-two patients, 11 men and women; median age 64 (range 38-83) years, median Karnofsky performance status 90% (range 50-100%), were included. Nineteen patients had primary IOL (PIOL): 13 a newly diagnosed disease and six an ocular relapse of primary central nervous system lymphoma (PCNSL). Three patients had secondary IOL. First-line treatment for IOL included systemic chemotherapy in 13 cases, ocular radiation in six and intraocular chemotherapy in three. Complete remission was achieved in 14/20 evaluable patients, partial remission in five and stable disease in one. All patients treated with ifosfamide (IFO) or trofosfamide (TRO) (n=8) responded. Median progression-free survival (PFS) and overall survival were 10 (range 1+ to 44.5+) and 22.5 (range 1+ to 49+) months, respectively. Patients with newly diagnosed PIOL and ocular relapse of PCNSL had a median PFS of 10 (range 1+ to 44.5+) and 6 (range 2 to 6+) months, respectively. Median PFS was 12 (range 3+ to 22.5+) months after systemic and 5.5 (range 1+ to 44.5+) months after local first-line therapy.
  
  
  CONCLUSIONS
  The prognosis of PIOL is similar to that of PCNSL without ocular involvement. Systemic therapy possibly prolongs PFS as compared with local management of (P)IOL. The high response rate to monotherapy with IFO and TRO is promising.
authors.affiliation: Department of Haematology, Oncology and Transfusion Medicine, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12200 Berlin, Germany. kristoph.jahnke@charite.de
authors.forename: Kristoph
authors.initials: K
authors.lastname: Jahnke
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Korfel
authors.forename: Julia
authors.initials: J
authors.lastname: Komm
authors.forename: Nikolaos E
authors.initials: NE
authors.lastname: Bechrakis
authors.forename: Harald
authors.initials: H
authors.lastname: Stein
authors.forename: Eckhard
authors.initials: E
authors.lastname: Thiel
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Coupland
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D003520:Cyclophosphamide / Q000031:analogs & derivatives / Q000627:therapeutic use; D005134:Eye Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D008223:Lymphoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D019572:Retinal Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D015996:Survival Rate; D014822:Vitreous Body / Q000473:pathology*
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003520:Cyclophosphamide; C003726:trofosfamide; D007069:Ifosfamide