Purpose

To describe the clinical presentation, treatment, and prognosis of natural killer- (NK) and T-cell lymphomas presenting in the ocular adnexa; i.e. the orbit, conjunctiva, eyelid, lacrimal gland and drainage system.

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Methods

Clinical data of patients with ocular adnexal NK- and T-cell lymphomas were retrospectively collected from five international eye cancer centers. Clinical data included age, sex, symptoms, clinical findings, staging, treatment, treatment response, and survival.

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Results

Ocular adnexal NK- and T-cell lymphomas were identified in 58 patients. The most frequent lymphoma subtypes were mycosis fungoides and Sézary syndrome (MF/SS) (n=13, 22%), extranodal NK/T-cell lymphoma (ENKTL) (n=12, 21%), peripheral T-cell lymphoma (not otherwise specified) (PTCL-NOS) (n=10, 17%), and primary cutaneous anaplastic large cell lymphoma (C-ALCL) (n=6, 10%). Patients were predominantly male (69%) with a median age of 59 years. For the full cohort, the 5-year overall survival was 53% (95%CI: 41-69), and the 5-year disease-specific mortality was 31% (95%CI: 19-44%). The eyelid was the most frequent ocular adnexal location (62%), due to a large proportion of primary cutaneous T-cell lymphomas (52%). Eyelid MF/SS presented primarily in elderly patients (median age 79 years), with a prior history of MF (77%), and advanced disease stage (54%). ENKTL frequently presented in the orbit (67%), and often with concurrent sinonasal disease (67%), and/or advanced disease stage (50%). PTCL-NOS presented primarily in younger adults (median age 34 years) with orbital disease (90%), and advanced stage in 40%. C-ALCL presented as a solitary eyelid tumor in 83% and had a favorable prognosis.

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Conclusion

This is the largest reported case-series of NK- and T-cell lymphomas with ocular adnexal presentation. Clinical presentation and patient prognosis varies between lymphoma subtypes. The eyelids are commonly involved, and patients are frequently males. This contrasts ocular adnexal B-cell lymphomas, which have a more equal gender distribution, and where the orbit and conjunctiva are more common locations.

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Additional Authors

First name	Last name	Base Hospital / Institution
Maja Dam	Andersen	Department of Hematology, Aarhus University Hospital, Aarhus, Denmark
Peter de Nully	Brown	Department of Hematology, Copenhagen University Hospital – Rigshospitalet, Copenhagen, Denmark
Sarah E.	Coupland	Liverpool Clinical Laboratories, Liverpool University Hospitals Foundation Trust, Liverpool, United Kingdom
Mathias	Dao	Department of Hematology, Aalborg University Hospital, Aalborg, Denmark
Bita	Esmaeli	The University of Texas M. D. Anderson Cancer Center, Houston, Texas. * Dr Esmaeli’s current affiliations are: Oncologic Ophthalmic Plastic & Orbital Surgery, LLC, Houston, Texas, USA; and Department of Clinical Sciences, University of Houston College of Medicine, Houston, Texas, USA
Santosh G.	Honavar	Department of Ocular Oncology and Oculoplastics, LV Prasad Eye Institute, Hyderabad, India; and Department of Ophthalmic and Facial Plastic Surgery, Orbit and Ocular Oncology, Centre for Sight, Hyderabad, India
Peter	Kamper	Department of Hematology, Aarhus University Hospital, Aarhus, Denmark
Jwu Jin	Khong	Orbital Plastic and Lacrimal Unit, The Royal Victorian Eye and Ear Hospital; Centre for Eye Research Australia; Department of Surgery, University of Melbourne, Melbourne, Australia
Jens Folke	Kiilgaard	Department of Ophthalmology, Copenhagen University Hospital – Rigshospitalet, Copenhagen, Denmark
Penny	McKelvie	Department of Anatomical Pathology, St Vincent’s Hospital Melbourne, Melbourne, Victoria, Australia
Kaustubh	Mulay	National Reporting Centre for Ophthalmic Pathology, Centre for Sight, Hyderabad, India
Marie Louise Roed	Rasmussen	Department of Ophthalmology, Copenhagen University Hospital – Rigshospitalet, Copenhagen, Denmark
Mikkel Straarup	Thagaard	Pathology Research Unit, Department of Pathology, Sygehus Sønderjylland, Aabenraa, Denmark; Institute for Regional Health Research, University of Southern Denmark, Odense, Denmark; and Department of Ophthalmology, Copenhagen University Hospital – Rigshospitalet, Copenhagen, Denmark
Carter	Tisdale	San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas, USA
Steffen	Heegaard	Department of pathology and department of ophthalmology, Copenhagen University Hospital – Rigshospitalet, Copenhagen, Denmark

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