Jaehne D, Coupland S E
Institut für Gewebediagnostik Pathologie MVZ, HELIOS Klinik Emil von Behring, Berlin, Deutschland.
Liverpool Ocular Oncology Research Group, Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, West Derby Street, L7 8TX, Liverpool, Großbritannien.
Ophthalmologe. 2018 Apr;115(4):343-356. doi: 10.1007/s00347-018-0681-5.
Primary vitreoretinal lymphoma (PVRL) is the most common intraocular lymphoma. It is a high grade malignant B‑cell lymphoma, which is thought to arise in the retina. It often infiltrates the central nervous system (CNS) and is therefore associated with a poor prognosis. The PVRL must be differentiated from other forms of intraocular lymphoma, such as the low-grade B‑cell lymphoma that rarely arises in the choroid. The choroidal lymphomas do not spread to the brain, they can be treated with low-dose external beam radiotherapy and the patients have a good prognosis. Since PVRL is a relatively rare tumor, there is little information with respect to its true incidence, to any geographical or ethnic variability and to the main risk factors apart from an association with immunosuppression, as a result of HIV or Epstein-Barr virus infections. The treatment of PVRL is very variable between oncology centres and is also dependent on the unilaterality or bilaterality of disease and whether there is any concomitant CNS involvement. Further studies and research projects in this field are necessary in order to diagnose PVRL at an early stage and to develop new targeted individualized treatment.
原发性玻璃体视网膜淋巴瘤（PVRL）是最常见的眼内淋巴瘤。它是一种高级别恶性B细胞淋巴瘤，被认为起源于视网膜。它常浸润中枢神经系统（CNS），因此预后较差。PVRL必须与其他形式的眼内淋巴瘤相鉴别，比如很少发生于脉络膜的低级别B细胞淋巴瘤。脉络膜淋巴瘤不会扩散至脑部，可用低剂量外照射放疗进行治疗，患者预后良好。由于PVRL是一种相对罕见的肿瘤，除了与因HIV或EB病毒感染导致的免疫抑制有关外，关于其真实发病率、任何地理或种族差异以及主要危险因素的信息很少。PVRL的治疗在不同肿瘤中心差异很大，还取决于疾病的单侧性或双侧性以及是否伴有CNS受累。为了早期诊断PVRL并开发新的靶向个体化治疗，该领域需要进一步的研究和科研项目。
