TY - JOUR
T1 - Clinicopathological features of ocular adnexal mantle-cell lymphoma in an international multicenter cohort
AU - Knudsen, Marina K.H.
AU - Rasmussen, Peter K.
AU - Coupland, Sarah E.
AU - Esmaeli, Bita
AU - Finger, Paul T.
AU - Graue, Gerardo F.
AU - Grossniklaus, Hans E.
AU - Khong, Jwu Jin
AU - McKelvie, Penny A.
AU - Mulay, Kaustubh
AU - Ralfkiaer, Elisabeth
AU - Sjö, Lene D.
AU - Vemuganti, Geeta K.
AU - Thuro, Bradley A.
AU - Curtin, Jeremy
AU - Heegaard, Steffen
N1 - Funding Information:
Funding/Support: This work was supported by grants from Fight for Sight Denmark, the Danish Cancer Society, the Danish Eye Research Foundation, Synoptik Foundation, the Danish Foundation for Cancer Research, Engineer Lars Andersens Foundation, the A. P. Møller Foundation for the Advancement of Medical Science, the Merchant Kjaer and Wife Kjaer, born la Cour-Holmens Foundation, and The Eye Cancer Foundation.
Publisher Copyright:
© 2017 American Medical Association. All rights reserved.
PY - 2017/12
Y1 - 2017/12
N2 - IMPORTANCE To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort. OBJECTIVE To characterize the clinical features of OA-MCL. DESIGN, SETTING, AND PARTICIPANTS This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months. MAIN OUTCOMES AND MEASURES Overall survival, disease-specific survival, and progression-free survival were the primary end points. RESULTS Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65%at 3 years (95%CI, 52%-78%) and 34%at 5 years (95%CI, 21%-47%). Disease-specific survival after 5 years was 38%for the entire cohort (95%CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95%CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95%CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95%CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region. CONCLUSIONS AND RELEVANCE These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.
AB - IMPORTANCE To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort. OBJECTIVE To characterize the clinical features of OA-MCL. DESIGN, SETTING, AND PARTICIPANTS This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months. MAIN OUTCOMES AND MEASURES Overall survival, disease-specific survival, and progression-free survival were the primary end points. RESULTS Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65%at 3 years (95%CI, 52%-78%) and 34%at 5 years (95%CI, 21%-47%). Disease-specific survival after 5 years was 38%for the entire cohort (95%CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95%CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95%CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95%CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region. CONCLUSIONS AND RELEVANCE These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.
UR - http://www.scopus.com/inward/record.url?scp=85039972430&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85039972430&partnerID=8YFLogxK
U2 - 10.1001/jamaophthalmol.2017.4810
DO - 10.1001/jamaophthalmol.2017.4810
M3 - Article
C2 - 29121219
AN - SCOPUS:85039972430
SN - 2168-6165
VL - 135
SP - 1367
EP - 1374
JO - JAMA Ophthalmology
JF - JAMA Ophthalmology
IS - 12
ER -