Oculoplastic and Orbital Surgery Service, Wills Eye Institute, Philadelphia, Pennsylvania 19107, USA.
Ophthalmic Plast Reconstr Surg. 2009 Nov-Dec;25(6):464-8. doi: 10.1097/IOP.0b013e3181b80fde.
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.
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.
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.
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.
描述 3 例散发型眼眶伯基特淋巴瘤的免疫功能正常患者，并复习文献中此类患者的结局。
回顾性分析病历和文献。排除免疫功能低下和非洲患者的眼眶伯基特淋巴瘤病例。测量的参数包括性别、年龄、眼部体征、影像学结果、全身受累范围和治疗。
当前病例和文献复习共纳入 16 例免疫功能正常的散发型眼眶伯基特淋巴瘤患者。发病时的中位年龄为 12 岁。最常见的眼部体征为眼球突出（13/16）、外展神经麻痹（9/16）和眼睑水肿（7/16）。16 例患者中有 5 例存在视神经病变。16 例患者中有 8 例存在副鼻窦累及。14 例（88%）患者存在全身受累，其中最常见的部位是中枢神经系统（6/16）、淋巴系统（6/16）、骨髓（6/16）和肝脏（4/16）。13 例患者的生存数据可用。7 例（54%）患者在发病后 12 个月内死亡。
散发型眼眶伯基特淋巴瘤发生于免疫功能正常的个体，年龄范围广泛。50%的患者伴有相邻副鼻窦受累。常见合并或随后发生的全身受累。文献复习中大量患者存在中枢神经系统受累，支持对所有眼眶伯基特淋巴瘤患者进行腰椎穿刺和神经影像学检查。预后仍不容乐观，发病后 1 年内死亡率较高。
