TY - JOUR

T1 - Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics

AU - Sjö, Lene D

AU - Heegaard, Steffen

AU - Prause, Jan U

AU - Petersen, Bodil L

AU - Pedersen, Sanni

AU - Ralfkiaer, Elisabeth

N1 - Keywords: Adaptor Proteins, Signal Transducing; Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Agents; Caspases; Child; Cytogenetics; Eye Neoplasms; Female; Genes, Immunoglobulin Heavy Chain; Humans; Immunophenotyping; In Situ Hybridization, Fluorescence; Lymphoma, B-Cell, Marginal Zone; Male; Middle Aged; Neoplasm Proteins; Prognosis; Radiotherapy; Survival Rate; Translocation, Genetic; Young Adult

PY - 2008

Y1 - 2008

N2 - 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 M Udgivelsesdato: 2009/2

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 M Udgivelsesdato: 2009/2

U2 - 10.1167/iovs.08-2539

DO - 10.1167/iovs.08-2539

M3 - Journal article

C2 - 18775860

VL - 50

SP - 516

EP - 522

JO - Investigative Ophthalmology & Visual Science

JF - Investigative Ophthalmology & Visual Science

SN - 0146-0404

IS - 2

ER -