Orbital burkitt lymphoma in immunocompetent patients: a report of 3 cases and a review of the literature.

Abstract:

PURPOSE: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

Baker PS,Gold KG,Lane KA,Bilyk JR,Katowitz JA

doi

10.1097/IOP.0b013e3181b80fde

subject

Has Abstract

pub_date

2009-11-01 00:00:00

pages

464-8

issue

6

eissn

0740-9303

issn

1537-2677

pii

00002341-200911000-00011

journal_volume

25

pub_type

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