Ocular adnexal diffuse large B-cell lymphoma: a multicenter international study.
OBJECTIVE
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).


METHODS
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.


METHODS
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
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.
Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark2Department of Pathology, Copenhagen University Hospital, Copenhagen, Denmark.
Helga D
HD
Munch-Petersen
Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark.
Peter K
PK
Rasmussen
Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, England.
Sarah E
SE
Coupland
Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston.
Bita
B
Esmaeli
The New York Eye Cancer Center, New York.
Paul T
PT
Finger
The New York Eye Cancer Center, New York.
Gerardo F
GF
Graue
Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia.
Hans E
HE
Grossniklaus
Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, and Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India.
Santosh G
SG
Honavar
Orbital, Plastic and Lacrimal Clinic, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
Jwu Jin
JJ
Khong
Orbital, Plastic and Lacrimal Clinic, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
Penny A
PA
McKelvie
National Reporting Centre for Ophthalmic Pathology, Centre for Sight, and Ocular Pathology, LV Prasad Eye Institute, Hyderabad, India.
Kaustubh
K
Mulay
Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark.
Jan U
JU
Prause
Department of Pathology, Copenhagen University Hospital, Copenhagen, Denmark.
Elisabeth
E
Ralfkiaer
Department of Pathology, Copenhagen University Hospital, Copenhagen, Denmark.
Lene D
LD
Sjö
Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston.
Matthew C
MC
Sniegowski
Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India11Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India.
Geeta K
GK
Vemuganti
Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark12Department of Ophthalmology, Glostrup University Hospital, Glostrup, Denmark.
Steffen
S
Heegaard
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D005134:Eye Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D014943:Global Health; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D055815:Young Adult