title: Lymphoma of the eyelid.
abstract: Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B-cell lymphoma (18 cases-9%). T-cell lymphomas are most frequently mycosis fungoides (25 cases-13%), extranodal natural killer/T-cell, nasal-type lymphoma (12 cases-6%), and primary cutaneous anaplastic large-cell lymphoma (12 cases-6%). This distribution differs from the distribution of ocular adnexal lymphoma and that of cutaneous lymphoma. The majority of subtypes occur in elderly patients, except for lymphoblastic lymphoma of B-cell and T-cell origin and Burkitt lymphoma, which occur in children and adolescents. Several subtypes have a male predominance, including peripheral T-cell lymphoma and Burkitt lymphoma. Only lymphomatoid papulosis has a female predominance. Signs of B-cell and T-cell lymphomas are tumor and swelling of the eyelid. Ulceration and erythema occur frequently among patients with T-cell lymphoma. Radiotherapy with or without surgery is the treatment of choice for low-grade, solitary lymphomas, whereas chemotherapy with or without adjuvant treatment is the treatment of choice for high-grade or disseminated lymphomas. The majority of subtypes, especially low-grade subtypes, have a good prognosis with few recurrences or progression. Some subtypes, including mycosis fungoides, have a poorer prognosis. Extranodal natural killer/T-cell lymphoma, nasal type has an exceedingly poor prognosis.
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; Department of Ophthalmology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. Electronic address: sthe@sund.ku.dk.
authors.forename: Steffen
authors.initials: S
authors.lastname: Heegaard
mesh_terms: D003131:Combined Modality Therapy; D005142:Eyelid Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D005143:Eyelids / Q000473:pathology*; D014943:Global Health; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D009017:Morbidity; D009367:Neoplasm Staging
keywords: characteristics; epidemiology; eyelid; lymphoma; pathology; survival; treatment