Ocular Adnexal Diffuse Large B-cell LymphomaA Multicenter International Study
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Ocular Adnexal Diffuse Large B-cell LymphomaA Multicenter International Study. / Munch-Petersen, Helga D; Rasmussen, Peter K; Coupland, Sarah E; Esmaeli, Bita; Finger, Paul T; Graue, Gerardo F; Grossniklaus, Hans E; Honavar, Santosh G; Khong, Jwu Jin; McKelvie, Penny A; Mulay, Kaustubh; Prause, Jan U; Ralfkiaer, Elisabeth; Sjö, Lene D; Sniegowski, Matthew C; Vemuganti, Geeta K; Heegaard, Steffen.
In: J A M A Ophthalmology, Vol. 133, No. 2, 02.2015, p. 165-73.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Ocular Adnexal Diffuse Large B-cell LymphomaA Multicenter International Study
AU - Munch-Petersen, Helga D
AU - Rasmussen, Peter K
AU - Coupland, Sarah E
AU - Esmaeli, Bita
AU - Finger, Paul T
AU - Graue, Gerardo F
AU - Grossniklaus, Hans E
AU - Honavar, Santosh G
AU - Khong, Jwu Jin
AU - McKelvie, Penny A
AU - Mulay, Kaustubh
AU - Prause, Jan U
AU - Ralfkiaer, Elisabeth
AU - Sjö, Lene D
AU - Sniegowski, Matthew C
AU - Vemuganti, Geeta K
AU - Heegaard, Steffen
PY - 2015/2
Y1 - 2015/2
N2 - IMPORTANCE: The clinical features of diffuse large B-cell lymphoma (DLBCL) subtype of ocular adnexal lymphoma have not previously been evaluated in a large cohort to our knowledge.OBJECTIVE: To investigate the clinical features of ocular adnexal DLBCL (OA-DLBCL).DESIGN, SETTING, AND PARTICIPANTS: This retrospective international cooperative study involved 6 eye cancer centers. During 30 years, 106 patients with OA-DLBCL were identified, and 6 were excluded from the study. The median follow-up period was 52 months.MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival (DSS), and progression-free survival were the primary end points.RESULTS: One hundred patients with OA-DLBCL were included in the study (median age, 70 years), of whom 54 (54.0%) were female. The following 3 groups of patients with lymphoma could be identified: primary OA-DLBCL (57.0%), OA-DLBCL and concurrent systemic lymphoma (29.0%), and ocular adnexal lymphoma relapse of previous systemic lymphoma (14.0%). Of 57 patients with primary OA-DLBCL, 53 (93.0%) had Ann Arbor stage IE disease, and 4 (7.0%) had Ann Arbor stage IIE disease. According to the TNM staging system, 43 of 57 (75.4%) had T2 tumors. Among all patients, the most frequent treatments were external beam radiation therapy with or without surgery (31.0%) and rituximab-cyclophosphamide, hydroxydaunorubicin, vincristine sulfate, prednisone (CHOP) or rituximab-CHOP-like chemotherapy with or without external beam radiation therapy (21.0%). The 5-year overall survival among the entire cohort was 36.0% (median, 3.5 years; 95% CI, 2.5-4.5 years). Relapse occurred in 43.9% (25 of 57) of patients with primary OA-DLBCL. Increasing T category of the TNM staging system was predictive of DSS (P = .04) in primary OA-DLBCL, whereas the Ann Arbor staging system was not. However, when taking all 100 patients into account, Ann Arbor stage was able to predict DSS (P = .01). Women had a longer median DSS than men (9.8 years; 95% CI, 1.9-17.7 years vs 3.3 years; 95% CI, 1.6-5.0; P = .03).CONCLUSIONS AND RELEVANCE: Most patients with primary OA-DLBCL were seen with Ann Arbor stage IE and TNM T2 disease. The 5-year overall survival was between 2.5 and 4.5 years, which is the 95% CI around the median of 3.5 years in this cohort. Increasing T category appears to be associated with decreased DSS among patients with primary OA-DLBCL. When taking all patients into account, sex and Ann Arbor stage also seem to be DSS predictors.
AB - IMPORTANCE: The clinical features of diffuse large B-cell lymphoma (DLBCL) subtype of ocular adnexal lymphoma have not previously been evaluated in a large cohort to our knowledge.OBJECTIVE: To investigate the clinical features of ocular adnexal DLBCL (OA-DLBCL).DESIGN, SETTING, AND PARTICIPANTS: This retrospective international cooperative study involved 6 eye cancer centers. During 30 years, 106 patients with OA-DLBCL were identified, and 6 were excluded from the study. The median follow-up period was 52 months.MAIN OUTCOMES AND MEASURES: Overall survival, disease-specific survival (DSS), and progression-free survival were the primary end points.RESULTS: One hundred patients with OA-DLBCL were included in the study (median age, 70 years), of whom 54 (54.0%) were female. The following 3 groups of patients with lymphoma could be identified: primary OA-DLBCL (57.0%), OA-DLBCL and concurrent systemic lymphoma (29.0%), and ocular adnexal lymphoma relapse of previous systemic lymphoma (14.0%). Of 57 patients with primary OA-DLBCL, 53 (93.0%) had Ann Arbor stage IE disease, and 4 (7.0%) had Ann Arbor stage IIE disease. According to the TNM staging system, 43 of 57 (75.4%) had T2 tumors. Among all patients, the most frequent treatments were external beam radiation therapy with or without surgery (31.0%) and rituximab-cyclophosphamide, hydroxydaunorubicin, vincristine sulfate, prednisone (CHOP) or rituximab-CHOP-like chemotherapy with or without external beam radiation therapy (21.0%). The 5-year overall survival among the entire cohort was 36.0% (median, 3.5 years; 95% CI, 2.5-4.5 years). Relapse occurred in 43.9% (25 of 57) of patients with primary OA-DLBCL. Increasing T category of the TNM staging system was predictive of DSS (P = .04) in primary OA-DLBCL, whereas the Ann Arbor staging system was not. However, when taking all 100 patients into account, Ann Arbor stage was able to predict DSS (P = .01). Women had a longer median DSS than men (9.8 years; 95% CI, 1.9-17.7 years vs 3.3 years; 95% CI, 1.6-5.0; P = .03).CONCLUSIONS AND RELEVANCE: Most patients with primary OA-DLBCL were seen with Ann Arbor stage IE and TNM T2 disease. The 5-year overall survival was between 2.5 and 4.5 years, which is the 95% CI around the median of 3.5 years in this cohort. Increasing T category appears to be associated with decreased DSS among patients with primary OA-DLBCL. When taking all patients into account, sex and Ann Arbor stage also seem to be DSS predictors.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Biopsy
KW - Child
KW - Child, Preschool
KW - Combined Modality Therapy
KW - Diagnosis, Differential
KW - Disease-Free Survival
KW - Eye Neoplasms
KW - Female
KW - Follow-Up Studies
KW - Global Health
KW - Humans
KW - Lymphoma, Large B-Cell, Diffuse
KW - Male
KW - Middle Aged
KW - Neoplasm Staging
KW - Prognosis
KW - Retrospective Studies
KW - Survival Rate
KW - Young Adult
U2 - 10.1001/jamaophthalmol.2014.4644
DO - 10.1001/jamaophthalmol.2014.4644
M3 - Journal article
C2 - 25393033
VL - 133
SP - 165
EP - 173
JO - JAMA Ophthalmology
JF - JAMA Ophthalmology
SN - 2168-6165
IS - 2
ER -
ID: 138898390