Paraneoplastic retinopathy

Shira S Simon MD MBA (

Dr. Simon of Northwestern University and Northwestern Memorial Hospital has research support from Quark, Inc.

)
Nicholas J Volpe MD (

Dr. Volpe of Northwestern University and Northwestern Memorial Hospital is an equity owner in Opticent Inc., has research support from Quark, Inc and has consulted for Opthotech.

)
Rimas V Lukas MD, editor. (

Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from AbbVie as a guest speaker, from NewLink Genetics and Reneuron for consulting work, and from Monetris as an advisory board member.

)
Originally released November 11, 1994; last updated April 16, 2019; expires April 16, 2022

Overview

Paraneoplastic disorders or remote effects of cancer can affect any part of the nervous system. Neurologists, oncologists, and ophthalmologists need to be aware that this includes the retina and optic nerve. Retinal neuronal dysfunction and degeneration may occur in association with a number of systemic neoplasms, including--most notably--melanoma and small cell lung carcinoma. For many patients, vision loss is the presenting feature of the associated tumor. Some affected patients have circulating antibodies against retinal antigens; these antibodies serve as diagnostic markers for the condition and may also play a role in causing retinal dysfunction. In this article, the authors summarize the clinical features, autoimmune pathogenesis, and treatment options for patients with paraneoplastic retinal degeneration.

Key points

 

• Paraneoplastic retinopathy is a rare entity associated with a variety of neoplasms, most commonly small cell lung carcinoma or melanoma.

 

• In most patients with cancer-associated paraneoplastic retinopathy, subacute vision loss is the presenting feature of the malignancy, whereas the majority of patients with melanoma-associated paraneoplastic retinopathy develop vision loss after the melanoma diagnosis.

 

• Pattern of vision loss and retinal dysfunction can vary depending on type of tumor and diagnosis is confirmed by widespread retinal dysfunction on electroretinogram (ERG).

 

• Most patients with paraneoplastic retinopathy have 1 or more antiretinal autoantibodies with varying immunohistochemical staining patterns and specificity for a number of retinal antigens.

 

• Immunosuppressive therapy should be offered in patients with vision loss from paraneoplastic retinopathy; early diagnosis may lead to a better outcome.

Historical note and terminology

The first well-documented cases of "photoreceptor degeneration as a remote effect of cancer" were reported in 1976 by Sawyer and colleagues (Sawyer et al 1976). The cases were of 3 female patients with bronchial carcinoma. The term "paraneoplastic autoimmune retinopathy" encompasses patients with heterogeneous tumor associations and clinical features and probably represents more than 1 pathophysiologic mechanism.

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