Marina K H Knudsen1, Peter K Rasmussen1, Sarah E Coupland2, Bita Esmaeli3, Paul T Finger4, Gerardo F Graue4, Hans E Grossniklaus5, Jwu Jin Khong6, Penny A McKelvie6, Kaustubh Mulay7, Elisabeth Ralfkiaer1, Lene D Sjö1, Geeta K Vemuganti8,9, Bradley A Thuro3, Jeremy Curtin6, Steffen Heegaard1,10. 1. Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark 2. Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, England 3. Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, University of Texas MD Anderson Cancer Center, Houston 4. The New York Eye Cancer Center, New York 5. Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia 6. Orbital, Plastic, and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia 7. National Reporting Centre for Ophthalmic Pathology, Centre for Sight, and Ocular Pathology, LV Prasad Eye Institute, Hyderabad, India 8. Visiting Faculty, Ophthalmic Pathology Services, LV Prasad Eye Institute, Hyderabad, India 9. School of Medical Sciences, University of Hyderabad, Hyderabad, India 10. Department of Ophthalmology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark
Abstract
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.
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.
Authors: Puneet Jain; Paul T Finger; Maria Fili; Bertil Damato; Sarah E Coupland; Heinrich Heimann; Nihal Kenawy; Niels J Brouwer; Marina Marinkovic; Sjoerd G Van Duinen; Jean Pierre Caujolle; Celia Maschi; Stefan Seregard; David Pelayes; Martin Folgar; Yacoub A Yousef; Hatem Krema; Brenda Gallie; Alberto Calle-Vasquez Journal: Br J Ophthalmol Date: 2020-09-05 Impact factor: 4.638