Grimm S A, McCannel C A, Omuro A M P, Ferreri A J M, Blay J-Y, Neuwelt E A, Siegal T, Batchelor T, Jahnke K, Shenkier T N, Hall A J, Graus F, Herrlinger U, Schiff D, Raizer J, Rubenstein J, Laperriere N, Thiel E, Doolittle N, Iwamoto F M, Abrey L E
Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Neurology. 2008 Oct 21;71(17):1355-60. doi: 10.1212/01.wnl.0000327672.04729.8c.
To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement.
A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma.
Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23%. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52%, eyes 19%, brain and eyes 12%, and systemic 2%. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p = 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months.
This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.
描述原发性中枢神经系统淋巴瘤（PCNSL）累及眼部患者的人口统计学特征、诊断细节、治疗管理及预后。
对来自7个国家16个中心的221例患者进行回顾性研究。仅纳入HIV阴性、免疫功能正常的脑和眼部淋巴瘤患者；均无全身性淋巴瘤。
诊断时的中位年龄为60岁。57%为女性。东部肿瘤协作组中位体能状态为2。眼部障碍和行为/认知改变是最常见的首发症状。淋巴瘤通过脑活检（147例）、玻璃体切除术（65例）或脑脊液细胞学检查（11例）确诊。眼内淋巴瘤通过玻璃体切除术/脉络膜/视网膜活检（90例）或临床眼科检查（141例）确诊。脑脊液细胞学检查23%呈阳性。176例患者有治疗信息。除脑淋巴瘤治疗外，共有102例接受了专门的眼部治疗（眼部放疗79例、玻璃体内注射甲氨蝶呤22例，两者均用1例）。69%的患者在中位时间13个月时病情进展；进展部位包括脑52%、眼19%、脑和眼12%、全身2%。接受局部眼部治疗的患者眼部失败风险无统计学显著降低（p = 0.7）。整个队列的中位无进展生存期和总生存期分别为18个月和31个月。
这是报道的原发性中枢神经系统淋巴瘤（PCNSL）累及眼内的最大系列病例。无进展生存期和总生存期与原发性中枢神经系统淋巴瘤报道的相似。专门的眼部治疗改善了疾病控制，但不影响总生存期。
