PDF(Pubmed)
Abstract：
Cancer-associated retinopathy (CAR) is a rare paraneoplastic syndrome characterized by autoimmune destruction of photoreceptor cells. It is associated with several tumor types, including small cell lung carcinoma (SCLC). Corticosteroids have been the mainstay treatment for CAR, although no therapeutic standard has truly been established. A 66-year-old female with significant smoking history and age-related macular degeneration (ARMD) presented with rapidly declining bilateral visual acuity. Ophthalmologic examination findings appeared consistent with the known diagnosis of ARMD but did not otherwise present a clear alternative etiology. Imaging with a computed tomography (CT) scan revealed a right hilar mass which was confirmed to be limited stage SCLC based on a subsequent biopsy and further imaging with a positron emission tomography/computed tomography (PET/CT) scan. Antibody testing was negative for anti-recoverin antibodies. The patient experienced a complete response to chemoradiation with cisplatin and etoposide; however, her ocular symptoms did not respond to a combined treatment approach with corticosteroids, plasmapheresis, and intravenous immunoglobulin (IVIG). While CAR represents a rare condition in SCLC, cases that are seronegative for anti-recoverin are even less common. Further, the diagnosis of CAR by ophthalmologic examination may be more challenging in patients with pre-existing ocular diseases, such as macular degeneration. Clinicians should have suspicion for paraneoplastic blindness in patients with known risk factors for malignancy, whose ocular symptoms are inconsistent with exam findings.
摘要：
癌症相关视网膜病变（CAR）是一种罕见的副肿瘤综合征，其特征是感光细胞的自身免疫破坏。它与几种肿瘤类型有关，包括小细胞肺癌（SCLC）。皮质类固醇一直是CAR的主要治疗方法，尽管尚未真正建立治疗标准。一名66岁女性，有明显的吸烟史和年龄相关性黄斑变性（ARMD），双侧视力迅速下降。眼科检查结果似乎与ARMD的已知诊断一致，但没有其他明确的替代病因。计算机断层扫描（CT）扫描成像显示右侧肺门肿块，根据随后的活检和正电子发射断层扫描/计算机断层扫描（PET/CT）扫描进一步成像，证实其为有限阶段的SCLC。抗体测试对于抗恢复素抗体是阴性的。患者对顺铂和依托泊苷的放化疗有完全反应；然而，她的眼部症状对皮质类固醇联合治疗没有反应，血浆置换,和静脉注射免疫球蛋白（IVIG）。虽然CAR在SCLC中代表一种罕见的情况，抗恢复蛋白血清阴性的情况甚至更不常见。Further,通过眼科检查对CAR的诊断在已经存在眼部疾病的患者中可能更具挑战性。如黄斑变性。在已知恶性肿瘤危险因素的患者中，临床医生应该怀疑副肿瘤性失明，其眼部症状与检查结果不一致。
