Cancer-associated Retinopathy Associated with Lung and Renal Cell Carcinoma: Paraneoplastic Visual Loss as the Initial Manifestation

Introduction

Cancer-associated retinopathy (CAR) is a complex and rare paraneoplastic syndrome. It is characterized by retinal dysfunction in the presence of an underlying malignancy. First described in the 1970s, CAR presents a spectrum of visual disturbances and electrophysiological abnormalities, making it a diagnostic challenge due to its diverse clinical manifestations and association with various malignancies. The pathogenesis of CAR involves immune-mediated mechanisms targeting retinal antigens, leading to retinal degeneration and visual impairment. Autoantibodies against retinal proteins, such as recoverin, alpha-enolase, and carbonic anhydrase II, have been implicated in the pathogenesis of CAR [1], [2]. These autoantibodies disrupt normal retinal function, resulting in photoreceptor dysfunction, abnormal electroretinography (ERG) findings, and progressive retinal degeneration [3]. However, a subset of cases presents without detectable antibodies, posing diagnostic challenges and prompting further investigation into its pathogenesis and management [4]. We present two such cases associated with lung and renal cell carcinoma, highlighting the need for early intervention and further research in this complex diagnosis.

Publication History

Received: 11 February 2025

Accepted: 26 May 2025

Accepted Manuscript online:
28 May 2025

Article published online:
15 August 2025

• References

• 1 Adamus G. Autoantibody targets and their cancer relationship in the pathogenicity of paraneoplastic retinopathy. Autoimmun Rev 2009; 8: 410-414
• 2 Ayhan Z, Unlu B, Seymenoglu G. et al. Cancer-associated retinopathy and its clinical features. Retina-Vitreus 2017; 26: 68-71
• 3 Heckenlively JR, Ferreyra HA. Autoimmune retinopathy: a review and summary. Semin Immunopathol 2008; 30: 127-134
• 4 Rahimy E, Sarraf D. Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: evaluation and management. Surv Ophthalmol 2013; 58: 430-458
• 5 Thomas M, Benfield J, Morales J. Case Report of Seronegative Cancer-Associated Retinopathy in a Patient with Small Cell Lung Carcinoma. Case Rep Oncol 2023; 16: 791-796
• 6 Wagley S, Tran TM, Mallory PW. et al. Cancer-Associated Retinopathy due to Clear Cell Renal Carcinoma. Ocul Oncol Pathol 2021; 7: 31-35
• 7 Oray M, Kir N, Tuncer S. et al. Autoimmune retinopathies: a report of 3 cases. Ocul Immunol Inflamm 2013; 21: 424-433
• 8 Lima LH, Greenberg JP, Greenstein VC. et al. Hyperautofluorescent ring in autoimmune retinopathy. Retina 2012; 32: 1385-1394
• 9 Adamus G, Champaigne R, Yang S. Occurrence of major anti-retinal autoantibodies associated with paraneoplastic autoimmune retinopathy. Clin Immunol 2020; 210: 108317
• 10 Kiratli H, Thirkill CE, Bilgic S. et al. Paraneoplastic retinopathy associated with metastatic cutaneous melanoma of unknown primary site. Eye (Lond) 1997; 11: 889-892
• 11 Brossard-Barbosa N, Dezard V, Margolin E. Treatment of Cancer-Associated Retinopathy: A Systematic Literature Review. Ophthalmol Retina 2023; 7: 819-828