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 - 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
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 -