Tine Gadegaard Olsen1, Frederik Holm2, Lauge Hjorth Mikkelsen3, Peter Kristian Rasmussen1, Sarah E Coupland4, Bita Esmaeli5, Paul T Finger6, Gerardo F Graue6, Hans E Grossniklaus7, Santosh G Honavar8, Jwu Jin Khong9, Penelope A McKelvie9, Kaustubh Mulay10, Lene Dissing Sjö2, Geeta K Vemuganti11, Bradley A Thuro5, Steffen Heegaard12. 1. Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 2. Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 3. Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 4. Department of Clinical and Cancer Medicine, University of Liverpool, Liverpool, England. 5. Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA. 6. The New York Eye Cancer Center, New York, New York, USA. 7. Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia, USA. 8. Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, Hyderabad, India; Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India. 9. Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia. 10. National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India. 11. Visiting Faculty, Ophthalmic Pathology Services, LV Prasad Eye Institute, Hyderabad, India; Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India. 12. Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. Electronic address: sthe@sund.ku.dk.
Abstract
PURPOSE: To investigate and characterize the clinical features of subtype-specific orbital lymphoma. DESIGN: Retrospective, interventional case series. METHODS: The study included 7 international eye cancer centers. Patient data were collected from January 1, 1980 through December 31, 2017. A total of 797 patients with a histologically verified orbital lymphoma were included. The primary endpoints were overall survival, disease-specific survival, and progression-free survival. RESULTS: The median age was 64 years, and 51% of patients (n = 407) were male. The majority of lymphomas were of B-cell origin (98%, n = 779). Extranodal marginal zone B-cell lymphoma (EMZL) was the most frequent subtype (57%, n = 452), followed by diffuse large B-cell lymphoma (DLBCL) (15%, n = 118), follicular lymphoma (FL) (11%, n = 91), and mantle cell lymphoma (MCL) (8%, n = 66). Localized Ann Arbor stage IE EMZL and FL were frequently treated with external beam radiation therapy. DLBCL, MCL, and disseminated EMZL and FL were primarily treated with chemotherapy. EMZL and FL patients had a markedly better prognosis (10-year disease-specific survival of 92% and 71%, respectively) than DLBCL and MCL patients (10-year disease-specific survival of 41% and 32%, respectively). CONCLUSIONS: Four lymphoma subtypes were primarily found in patients with orbital lymphoma: EMZL, DLBCL, FL, and MCL. The histologic subtype was found to be the main predictor for outcome, with EMZL and FL patients having a markedly better prognosis than DLBCL and MCL.
PURPOSE: To investigate and characterize the clinical features of subtype-specific orbital lymphoma. DESIGN: Retrospective, interventional case series. METHODS: The study included 7 international eye cancer centers. Patient data were collected from January 1, 1980 through December 31, 2017. A total of 797 patients with a histologically verified orbital lymphoma were included. The primary endpoints were overall survival, disease-specific survival, and progression-free survival. RESULTS: The median age was 64 years, and 51% of patients (n = 407) were male. The majority of lymphomas were of B-cell origin (98%, n = 779). Extranodal marginal zone B-cell lymphoma (EMZL) was the most frequent subtype (57%, n = 452), followed by diffuse large B-cell lymphoma (DLBCL) (15%, n = 118), follicular lymphoma (FL) (11%, n = 91), and mantle cell lymphoma (MCL) (8%, n = 66). Localized Ann Arbor stage IE EMZL and FL were frequently treated with external beam radiation therapy. DLBCL, MCL, and disseminated EMZL and FL were primarily treated with chemotherapy. EMZL and FL patients had a markedly better prognosis (10-year disease-specific survival of 92% and 71%, respectively) than DLBCL and MCLpatients (10-year disease-specific survival of 41% and 32%, respectively). CONCLUSIONS: Four lymphoma subtypes were primarily found in patients with orbital lymphoma: EMZL, DLBCL, FL, and MCL. The histologic subtype was found to be the main predictor for outcome, with EMZL and FL patients having a markedly better prognosis than DLBCL and MCL.
Authors: Marco Zschoche; Annette Zimpfer; Björn O Scheef; Anselm M Jünemann; Rudolf F Guthoff; Christian Junghanss; Guido Hildebrandt; Steffen Emmert; Andreas Erbersdobler; Günther Kundt; Vinodh Kakkassery Journal: In Vivo Date: 2020 Jul-Aug Impact factor: 2.155
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
Authors: Kamil G Laban; Rachel Kalmann; Cornelis P J Bekker; Sanne Hiddingh; Rob L P van der Veen; Christine A E Eenhorst; Stijn W Genders; Maarten P Mourits; Fleurieke H Verhagen; Emmerik F A Leijten; Saskia Haitjema; Mark C H de Groot; Timothy R D J Radstake; Joke H de Boer; Jonas J W Kuiper Journal: Eur J Immunol Date: 2019-11-25 Impact factor: 5.532