Sobottka B, Schlote T, Besch D, Djelebova T, Wilhelm H, Zrenner E
Abteilung III, Univ.-Augenklinik Tübingen.
Klin Monbl Augenheilkd. 2000 Jan;216(1):17-24. doi: 10.1055/s-2000-10511.
Cancer-associated retinopathy (CAR) is a rare paraneoplastic syndrome. In this survey we report about two further patients with CAR, who were referred to the University Eye Hospital of Tuebingen within a few months. The most common primary tumor associated with CAR is small cell carcinoma of the lung. Case reports about rhabdomyosarcoma, carcinoma of the endometrium, prostate and mamma were also described. The exact pathogenesis of CAR is still unknown. Specific autoantibodies were found against the photoreceptor protein recovering (23-kd retinal CAR antigen). However, this reaction is not present in all patients, and probably other antigens are also involved. Most of the patients experience symptoms of CAR before the primary tumor is detected. Besides glare sensitivity and flashing lights, a rapidly progressive, often asymmetric visual loss may occur. Although paracentral and mid-peripheral scotomas can be found frequently, visual field defects are often quite heterogeneous. Typically, the responses in the electroretinogram (ERG) are markedly reduced, but normal ERGs were also described. The fundus picture in CAR shows sheathing of the retinal vessels, narrowing of the arterioles and clumbing of the retinal pigment epithelium. The prognosis is poor. Frequently there is progression to bilateral loss of vision within a few months. Treatment of the primary tumor does not seem to alter the ocular prognosis. Systemic corticosteroids may be helpful in some patients. Nevertheless, no proven therapeutic regimen is currently available.
癌症相关性视网膜病变(CAR)是一种罕见的副肿瘤综合征。在本次调查中,我们报告了另外两名患有CAR的患者,他们在几个月内被转诊至图宾根大学眼科医院。与CAR相关的最常见原发性肿瘤是肺小细胞癌。也有关于横纹肌肉瘤、子宫内膜癌、前列腺癌和乳腺癌的病例报告。CAR的确切发病机制仍不清楚。已发现针对光感受器蛋白恢复蛋白(23-kd视网膜CAR抗原)的特异性自身抗体。然而,并非所有患者都存在这种反应,可能还涉及其他抗原。大多数患者在原发性肿瘤被检测到之前就出现了CAR症状。除了畏光和闪光外,还可能出现快速进展、通常不对称的视力丧失。虽然经常可以发现旁中心和中周边暗点,但视野缺损往往非常不均匀。典型地,视网膜电图(ERG)反应明显降低,但也有ERG正常的描述。CAR的眼底表现为视网膜血管鞘、小动脉变窄和视网膜色素上皮增生。预后很差。通常在几个月内会进展为双侧视力丧失。原发性肿瘤的治疗似乎不会改变眼部预后。全身用皮质类固醇对一些患者可能有帮助。然而,目前尚无经证实的治疗方案。