International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma
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International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma. / Hindsø, Tine Gadegaard; Esmaeli, Bita; Holm, Frederik; Mikkelsen, Lauge Hjorth; Rasmussen, Peter Kristian; Coupland, Sarah E.; Finger, Paul T.; Graue, Gerardo F.; Grossniklaus, Hans E.; Honavar, Santosh G.; Khong, Jwu Jin; McKelvie, Penelope A.; Mulay, Kaustubh; Sjö, Lene Dissing; Vemuganti, Geeta K.; Thuro, Bradley A.; Heegaard, Steffen.
In: British Journal of Ophthalmology, Vol. 104, No. 3, 03.2020, p. 357-362.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - International multicentre retrospective cohort study of ocular adnexal marginal zone B-cell lymphoma
AU - Hindsø, Tine Gadegaard
AU - Esmaeli, Bita
AU - Holm, Frederik
AU - Mikkelsen, Lauge Hjorth
AU - Rasmussen, Peter Kristian
AU - Coupland, Sarah E.
AU - Finger, Paul T.
AU - Graue, Gerardo F.
AU - Grossniklaus, Hans E.
AU - Honavar, Santosh G.
AU - Khong, Jwu Jin
AU - McKelvie, Penelope A.
AU - Mulay, Kaustubh
AU - Sjö, Lene Dissing
AU - Vemuganti, Geeta K.
AU - Thuro, Bradley A.
AU - Heegaard, Steffen
PY - 2020/3
Y1 - 2020/3
N2 - Backgrounds/aims To date, this is the largest cohort study on extranodal marginal zone B-cell lymphoma (EMZL) of the ocular adnexa (OA). The aim of the study was to characterise the clinical features of OA-EMZL. Methods A retrospective multicentre study involving seven international eye cancer centres. Data were collected from 1 January 1980 through 31 December 2017. A total of 689 patients with OA-EMZL were included. Results The median follow-up time was 42 months. The median age was 62 years (range, 8-100 years), and 55 % (378/689 patients) of patients were women. The majority of patients (82%, 558/680 patients) were diagnosed with primary OA-EMZL with Ann Arbor stage IE (90%, 485/541 patients) and American Joint Committee on Cancer stage T2 (61%, 340/557 patients) at the time of diagnosis. The orbit (66%, 452/689 patients) and the conjunctiva (37%, 255/689 patients) were the most frequently involved anatomical structures. The 5-year, 10-year and 20-year disease-specific survival (DSS) were 96%, 91% and 90%, respectively. Stage IE patients treated with external beam radiation therapy (EBRT) as monotherapy (10-year DSS, 95%) were found to have a better DSS than stage IE patients treated with chemotherapy (10-year DSS, 86%). Stage IIIE/IVE patients treated with chemotherapy and rituximab had a better DSS (10-year DSS, 96%) than stage IIIE/IVE patients treated with chemotherapy without rituximab (10-year DSS, 63%). Conclusions and relevance EMZL is a slow-growing tumour with an excellent long-term survival. Low-dose EBRT as monotherapy should be considered in localised OA-EMZL. Rituximab-based chemotherapy should be chosen in those patients with disseminated disease.
AB - Backgrounds/aims To date, this is the largest cohort study on extranodal marginal zone B-cell lymphoma (EMZL) of the ocular adnexa (OA). The aim of the study was to characterise the clinical features of OA-EMZL. Methods A retrospective multicentre study involving seven international eye cancer centres. Data were collected from 1 January 1980 through 31 December 2017. A total of 689 patients with OA-EMZL were included. Results The median follow-up time was 42 months. The median age was 62 years (range, 8-100 years), and 55 % (378/689 patients) of patients were women. The majority of patients (82%, 558/680 patients) were diagnosed with primary OA-EMZL with Ann Arbor stage IE (90%, 485/541 patients) and American Joint Committee on Cancer stage T2 (61%, 340/557 patients) at the time of diagnosis. The orbit (66%, 452/689 patients) and the conjunctiva (37%, 255/689 patients) were the most frequently involved anatomical structures. The 5-year, 10-year and 20-year disease-specific survival (DSS) were 96%, 91% and 90%, respectively. Stage IE patients treated with external beam radiation therapy (EBRT) as monotherapy (10-year DSS, 95%) were found to have a better DSS than stage IE patients treated with chemotherapy (10-year DSS, 86%). Stage IIIE/IVE patients treated with chemotherapy and rituximab had a better DSS (10-year DSS, 96%) than stage IIIE/IVE patients treated with chemotherapy without rituximab (10-year DSS, 63%). Conclusions and relevance EMZL is a slow-growing tumour with an excellent long-term survival. Low-dose EBRT as monotherapy should be considered in localised OA-EMZL. Rituximab-based chemotherapy should be chosen in those patients with disseminated disease.
KW - conjunctiva
KW - epidemiology
KW - eye lids
KW - neoplasia
KW - orbit
U2 - 10.1136/bjophthalmol-2019-314008
DO - 10.1136/bjophthalmol-2019-314008
M3 - Journal article
C2 - 31177189
AN - SCOPUS:85067029717
VL - 104
SP - 357
EP - 362
JO - British Journal of Ophthalmology
JF - British Journal of Ophthalmology
SN - 0007-1161
IS - 3
ER -
ID: 240690683