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Case Report of Seronegative Cancer-Associated Retinopathy in a Patient with Small Cell Lung Carcinoma
Miles Thomas1, John Benfield2, Joshua Morales3
1Internal Medicine, Virginia Tech Carilion School of Medicine, Roanoke, VA, USA.
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.
Insights
Cancer-associated retinopathy (CAR), a rare paraneoplastic syndrome, can occur with small cell lung cancer (SCLC). This case highlights diagnostic challenges in patients with pre-existing eye conditions, even without typical antibodies.
Area of Science:
- Ophthalmology
- Oncology
- Immunology
Background:
- Cancer-associated retinopathy (CAR) is a rare autoimmune paraneoplastic syndrome affecting photoreceptor cells.
- It is frequently associated with small cell lung carcinoma (SCLC), but treatment standards remain undefined.
- Age-related macular degeneration (ARMD) can complicate CAR diagnosis.
Observation:
- A 66-year-old female with smoking history and ARMD presented with rapidly declining vision.
- CT and PET/CT scans revealed limited-stage SCLC.
- Ophthalmologic findings mimicked ARMD, and antibody testing was negative for anti-recoverin.
Findings:
- The patient achieved a complete response to SCLC chemoradiation but showed no improvement in ocular symptoms despite treatment with corticosteroids, plasmapheresis, and IVIG.
- This case represents a rare instance of CAR in SCLC that is seronegative for anti-recoverin antibodies.
- Diagnosing CAR is challenging in patients with concurrent ocular diseases like ARMD.
Implications:
- Clinicians must maintain a high suspicion for paraneoplastic visual loss in patients with malignancy risk factors and atypical ocular symptoms.
- Early recognition and management of CAR, even in seronegative cases, are crucial for potentially preserving vision.
- This case underscores the importance of a multidisciplinary approach in managing complex paraneoplastic syndromes.
