PMID- 18775860 OWN - NLM STAT- MEDLINE DCOM- 20090206 LR - 20220409 IS - 1552-5783 (Electronic) IS - 0146-0404 (Linking) VI - 50 IP - 2 DP - 2009 Feb TI - Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics. PG - 516-22 LID - 10.1167/iovs.08-2539 [doi] AB - PURPOSE: To evaluate clinical, immunophenotypical, and cytogenetical characteristics of 116 patients with a diagnosis of extranodal marginal zone lymphoma (EMZL) presenting primarily in the ocular region. METHODS: Specimens from all patients with a diagnosis of ophthalmic lymphoma in Denmark during the period 1980 to 2005 were reviewed and reclassified according to the World Health Organization (WHO) classification. Cases reclassified as EMZL were selected and reviewed with respect to clinical characteristics and outcome. The presence of translocations involving IGH and/or MALT1 was investigated in 42 specimens by fluorescence in situ hybridization (FISH). RESULTS: Median age was 69 years. Most lymphomas were located in the orbit. Approximately one fourth of the patients had disseminated disease at presentation. One third experienced a relapse or progression of disease after initial therapy, and relapses were frequently found at extraocular sites. Five-year progression-free survival and overall survival (OS) rates were 71% and 75%, respectively. Translocations involving the IGH- or MALT1-gene loci were detected in 2 (5%) of 42 specimens. In Cox regression multivariate analysis, IGH-translocation was the only factor associated with PFS, whereas a favorable International Prognostic Index (IPI) score was the most reliable predictor of OS. CONCLUSIONS: EMZL presenting in the ocular region usually runs an indolent course, but relapses are frequently seen. The IPI-score was the most reliable independent parameter for estimating risk of death in our cohort of patients. Furthermore, we found that the frequency of translocations involving the MALT1- and IGH-gene loci is low in ocular region EMZL. FAU - Sjo, Lene D AU - Sjo LD AD - Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark. FAU - Heegaard, Steffen AU - Heegaard S FAU - Prause, Jan U AU - Prause JU FAU - Petersen, Bodil L AU - Petersen BL FAU - Pedersen, Sanni AU - Pedersen S FAU - Ralfkiaer, Elisabeth AU - Ralfkiaer E LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20080904 PL - United States TA - Invest Ophthalmol Vis Sci JT - Investigative ophthalmology & visual science JID - 7703701 RN - 0 (Adaptor Proteins, Signal Transducing) RN - 0 (Antineoplastic Agents) RN - 0 (B-Cell CLL-Lymphoma 10 Protein) RN - 0 (BCL10 protein, human) RN - 0 (Neoplasm Proteins) RN - EC 3.4.22.- (Caspases) RN - EC 3.4.22.- (MALT1 protein, human) RN - EC 3.4.22.- (Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein) SB - IM MH - Adaptor Proteins, Signal Transducing/genetics MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Antineoplastic Agents/therapeutic use MH - B-Cell CLL-Lymphoma 10 Protein MH - Caspases/genetics MH - Child MH - Cytogenetics MH - Eye Neoplasms/*genetics/mortality/*pathology/therapy MH - Female MH - Genes, Immunoglobulin Heavy Chain/genetics MH - Humans MH - Immunophenotyping MH - In Situ Hybridization, Fluorescence MH - Lymphoma, B-Cell, Marginal Zone/*genetics/mortality/*pathology/therapy MH - Male MH - Middle Aged MH - Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein MH - Neoplasm Proteins/genetics MH - Prognosis MH - Radiotherapy MH - Survival Rate MH - Translocation, Genetic MH - Young Adult EDAT- 2008/09/09 09:00 MHDA- 2009/02/07 09:00 CRDT- 2008/09/09 09:00 PHST- 2008/09/09 09:00 [pubmed] PHST- 2009/02/07 09:00 [medline] PHST- 2008/09/09 09:00 [entrez] AID - iovs.08-2539 [pii] AID - 10.1167/iovs.08-2539 [doi] PST - ppublish SO - Invest Ophthalmol Vis Sci. 2009 Feb;50(2):516-22. doi: 10.1167/iovs.08-2539. Epub 2008 Sep 4.