title: Orbital burkitt lymphoma in immunocompetent patients: a report of 3 cases and a review of the literature.
abstract: OBJECTIVE
  To describe a series of 3 immunocompetent patients with the sporadic form of orbital Burkitt lymphoma and review the outcomes of such patients reported in the scientific literature.
  
  
  METHODS
  Retrospective review of medical records and the literature. Cases of orbital Burkitt lymphoma in immunocompromised and African patients were excluded from the review. Measured parameters included gender, age, ocular signs, imaging results, the range of systemic involvement, and treatment.
  
  
  RESULTS
  Current cases and review of the scientific literature resulted in 16 immunocompetent patients with sporadic orbital Burkitt lymphoma. The median age at presentation was 12 years. Most common ocular signs at presentation were proptosis (13/16), external ophthalmoplegia (9/16), and eyelid edema (7/16). Optic neuropathy was noted in 5 of 16 patients. Concomitant paranasal sinus involvement was present in 8 of 16 patients. Fourteen (88%) of the patients had systemic involvement, of which the most common locations were central nervous system (6/16), lymphatics (6/16), bone marrow (6/16), and liver (4/16). Survival data were available for 13 patients. Seven patients (54%) died within 12 months of presentation.
  
  
  CONCLUSIONS
  Sporadic orbital Burkitt lymphoma occurs in immunocompetent individuals with a wide age range. Fifty percent presented with adjacent paranasal sinus involvement. Concurrent or eventual systemic involvement is common. The large number of patients with central nervous system involvement in our review of the literature supports the role of lumbar puncture and neuroimaging in the workup of all patients with orbital Burkitt lymphoma. Prognosis remains guarded, with significant mortality within 1 year of presentation.
authors.affiliation: Oculoplastic and Orbital Surgery Service, Wills Eye Institute, Philadelphia, Pennsylvania 19107, USA. pbakerny@yahoo.com
authors.forename: Paul S
authors.initials: PS
authors.lastname: Baker
authors.forename: Katherine G
authors.initials: KG
authors.lastname: Gold
authors.forename: Katherine A
authors.initials: KA
authors.lastname: Lane
authors.forename: Jurij R
authors.initials: JR
authors.lastname: Bilyk
authors.forename: James A
authors.initials: JA
authors.lastname: Katowitz
mesh_terms: D000368:Aged; D002051:Burkitt Lymphoma / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D010255:Paranasal Sinus Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed