Frederik Holm Svendsen1, Peter Kristian Rasmussen1, Sarah E Coupland2, Bita Esmaeli3, Paul T Finger4, Gerardo F Graue4, Hans E Grossniklaus5, Santosh G Honavar6, Jwu Jin Khong7, Penelope A McKelvie7, Kaustubh Mulay8, Elisabeth Ralfkiaer1, Lene Dissing Sjö1, Geeta K Vemuganti9, Bradley A Thuro3, Jeremy Curtin7, Steffen Heegaard10. 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, The University of Texas MD Anderson Cancer Center, Houston, Texas. 4. The New York Eye Cancer Center, New York, New York. 5. Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia. 6. Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, Hyderabad, India; Department of Ocular Oncology and Oculoplastics, L V Prasad Eye Institute, Hyderabad, India. 7. Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia. 8. National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India. 9. Ophthalmic Pathology Services, L V Prasad Eye Institute, Hyderabad, India; Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India. 10. Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Ophthalmology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark. Electronic address: sthe@sund.ku.dk.
Abstract
PURPOSE: To document subtype-specific clinical features of lymphoma of the eyelid, and their effect on patient outcome. DESIGN: Retrospective observational case series. METHODS: Patient data were collected from 7 international eye cancer centers from January 1, 1980 through December 31, 2015. The cases included primary and secondary lymphomas affecting the eyelid. Overall survival, disease-specific survival (DSS), and progression-free survival were the primary endpoints. RESULTS: Eighty-six patients were included. Mean age was 63 years and 47 (55%) were male. Non-Hodgkin B-cell lymphomas constituted 83% (n = 71) and T-cell lymphomas constituted 17% (n = 15). The most common subtypes were extranodal marginal-zone lymphoma (EMZL) (37% [n = 32]), follicular lymphoma (FL) (23% [n = 20]), diffuse large B-cell lymphoma (DLBCL) (10% [n = 9]), mantle cell lymphoma (MCL) (8% [n = 7]), and mycosis fungoides (MF) (9% [n = 8]). EMZL had a female predilection (69% [22 of 32]), whereas MCL (71% [5 of 7]) and MF (88% [7 of 8]) had a male predominance. MCL (57% [4 of 7]), DLBCL (56% [5 of 9]), and MF (88% [7 of 8]) were frequently secondary lymphomas. Localized EMZL and FL were mostly treated with external beam radiation therapy, whereas DLBCL, MCL, and high Ann Arbor stage EMZL and FL were frequently treated with chemotherapy. DLBCL and MCL had a poor prognosis (5-year DSS, 21% and 50%, respectively), whereas EMZL, FL, and MF had a good prognosis (5-year DSS, 88%, 88% and 86%, respectively). CONCLUSIONS: Lymphoma of the eyelid consists mainly of the lymphoma subtypes EMZL, FL, DLBCL, MCL, and MF. High-grade DLBCL and MCL, as well as MF, are frequently secondary eyelid lymphomas. The main predictor of outcome was the histologic subtype: EMZL, FL, and MF had a significantly better prognosis than MCL and DLBCL.
PURPOSE: To document subtype-specific clinical features of lymphoma of the eyelid, and their effect on patient outcome. DESIGN: Retrospective observational case series. METHODS:Patient data were collected from 7 international eye cancer centers from January 1, 1980 through December 31, 2015. The cases included primary and secondary lymphomas affecting the eyelid. Overall survival, disease-specific survival (DSS), and progression-free survival were the primary endpoints. RESULTS: Eighty-six patients were included. Mean age was 63 years and 47 (55%) were male. Non-Hodgkin B-cell lymphomas constituted 83% (n = 71) and T-cell lymphomas constituted 17% (n = 15). The most common subtypes were extranodal marginal-zone lymphoma (EMZL) (37% [n = 32]), follicular lymphoma (FL) (23% [n = 20]), diffuse large B-cell lymphoma (DLBCL) (10% [n = 9]), mantle cell lymphoma (MCL) (8% [n = 7]), and mycosis fungoides (MF) (9% [n = 8]). EMZL had a female predilection (69% [22 of 32]), whereas MCL (71% [5 of 7]) and MF (88% [7 of 8]) had a male predominance. MCL (57% [4 of 7]), DLBCL (56% [5 of 9]), and MF (88% [7 of 8]) were frequently secondary lymphomas. Localized EMZL and FL were mostly treated with external beam radiation therapy, whereas DLBCL, MCL, and high Ann Arbor stage EMZL and FL were frequently treated with chemotherapy. DLBCL and MCL had a poor prognosis (5-year DSS, 21% and 50%, respectively), whereas EMZL, FL, and MF had a good prognosis (5-year DSS, 88%, 88% and 86%, respectively). CONCLUSIONS:Lymphoma of the eyelid consists mainly of the lymphoma subtypes EMZL, FL, DLBCL, MCL, and MF. High-grade DLBCL and MCL, as well as MF, are frequently secondary eyelid lymphomas. The main predictor of outcome was the histologic subtype: EMZL, FL, and MF had a significantly better prognosis than MCL and DLBCL.
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