Joubert R, Bonnin N, Kemeny J-L, Moluçon-Chabrot C, Tournilhac O, Bacin F, Chiambaretta F
Service d'ophtalmologie, pôle médecine interne-ophtalmologie-ORL, CHU de Clermont-Ferrand, 58, rue Montalembert, 63000 Clermont-Ferrand, France.
Service d'ophtalmologie, pôle médecine interne-ophtalmologie-ORL, CHU de Clermont-Ferrand, 58, rue Montalembert, 63000 Clermont-Ferrand, France; EA 7281 R2D2, Biochemistry Laboratory, Medicine Faculty, Auvergne University, 28, place Henri-Dunant, 63001 Clermont-Ferrand, France.
J Fr Ophtalmol. 2015 Jun;38(6):559-66. doi: 10.1016/j.jfo.2015.03.005. Epub 2015 May 11.
Primary intraocular lymphoma (PIOL), associated with primary central nervous system lymphoma (PCNSL), is a rare malignancy disease. By way of a seven-year experience of a tertiary center, we discuss the presentation and we review the diagnostic and therapeutic modalities.
We report six cases of PIOL associated with PCNSL. For all patients, the clinical presentation was a vitreoretinal syndrome. The diagnosis was histologically confirmed by vitreal sample or brain biopsy. Five patients developed a diffuse large B-cell lymphoma. Only one patient developed a T-cell lymphoma. The treatment consisted of conformational radiation therapy, systemic chemotherapy and intravitreal injections of methotrexate. The median survival after the diagnosis was 24 months.
PIOL, associated with PCNSL, is the most common type of ocular lymphoma. In most cases, ocular manifestations inaugurate the disease. PIOL is often fatal because of ultimate central nervous system presentation. The role of the ophthalmologist consists in early diagnosis. Typical clinical findings include vitroretinal tumor syndrome but can mascarade other eye pathologies. Diagnosis requires histology. The majority of PIOL is diffused large B-cell lymphoma. Decisions are made through multidisciplinary consultation. PIOL exhibits high responsiveness to methotrexate.
Through a literature review and many illustrations, we discuss epidemiological, clinical, histological, radiological and treatment characteristics of PIOL associated with PCNSL.
原发性眼内淋巴瘤（PIOL）与原发性中枢神经系统淋巴瘤（PCNSL）相关，是一种罕见的恶性疾病。通过一家三级中心的七年经验，我们讨论其临床表现，并回顾诊断和治疗方式。
我们报告了6例与PCNSL相关的PIOL病例。所有患者的临床表现均为玻璃体视网膜综合征。诊断通过玻璃体样本或脑活检进行组织学确认。5例患者发展为弥漫性大B细胞淋巴瘤。只有1例患者发展为T细胞淋巴瘤。治疗包括适形放疗、全身化疗和玻璃体内注射甲氨蝶呤。诊断后的中位生存期为24个月。
与PCNSL相关的PIOL是最常见的眼内淋巴瘤类型。在大多数情况下，眼部表现是疾病的首发症状。PIOL通常因最终出现中枢神经系统症状而致命。眼科医生的作用在于早期诊断。典型的临床发现包括玻璃体视网膜肿瘤综合征，但可能掩盖其他眼部疾病。诊断需要组织学检查。大多数PIOL是弥漫性大B细胞淋巴瘤。决策通过多学科会诊做出。PIOL对甲氨蝶呤表现出高反应性。
通过文献综述和众多实例，我们讨论了与PCNSL相关的PIOL的流行病学、临床、组织学、放射学和治疗特征。
