title: Lymphoma of the Eyelid - An International Multicenter Retrospective Study.
abstract: OBJECTIVE
  To document subtype-specific clinical features of lymphoma of the eyelid, and their effect on patient outcome.
  
  
  METHODS
  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.affiliation: Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Frederik Holm
authors.initials: FH
authors.lastname: Svendsen
authors.affiliation: Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Peter Kristian
authors.initials: PK
authors.lastname: Rasmussen
authors.affiliation: Department of Cellular and Molecular Pathology, University of Liverpool, Liverpool, England.
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Coupland
authors.affiliation: Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Bita
authors.initials: B
authors.lastname: Esmaeli
authors.affiliation: The New York Eye Cancer Center, New York, New York.
authors.forename: Paul T
authors.initials: PT
authors.lastname: Finger
authors.affiliation: The New York Eye Cancer Center, New York, New York.
authors.forename: Gerardo F
authors.initials: GF
authors.lastname: Graue
authors.affiliation: Section of Ocular Oncology, Emory Eye Center, Atlanta, Georgia.
authors.forename: Hans E
authors.initials: HE
authors.lastname: Grossniklaus
authors.affiliation: 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.
authors.forename: Santosh G
authors.initials: SG
authors.lastname: Honavar
authors.affiliation: Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
authors.forename: Jwu Jin
authors.initials: JJ
authors.lastname: Khong
authors.affiliation: Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
authors.forename: Penelope A
authors.initials: PA
authors.lastname: McKelvie
authors.affiliation: National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India.
authors.forename: Kaustubh
authors.initials: K
authors.lastname: Mulay
authors.affiliation: Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Ralfkiaer
authors.affiliation: Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
authors.forename: Lene Dissing
authors.initials: LD
authors.lastname: Sjö
authors.affiliation: Ophthalmic Pathology Services, L V Prasad Eye Institute, Hyderabad, India; Kallam Anji Reddy Campus, School of Medical Sciences, University of Hyderabad, Hyderabad, India.
authors.forename: Geeta K
authors.initials: GK
authors.lastname: Vemuganti
authors.affiliation: Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Bradley A
authors.initials: BA
authors.lastname: Thuro
authors.affiliation: Orbital, Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Curtin
authors.affiliation: 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.
authors.forename: Steffen
authors.initials: S
authors.lastname: Heegaard
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001706:Biopsy; D003718:Denmark / Q000453:epidemiology; D018572:Disease-Free Survival; D004739:England / Q000453:epidemiology; D005142:Eyelid Neoplasms / Q000175:diagnosis / Q000453:epidemiology*; D005143:Eyelids / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007194:India / Q000453:epidemiology; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000453:epidemiology*; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D014739:Victoria / Q000453:epidemiology