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  • Updated 04.06.2020
  • Released 11.11.1994
  • Expires For CME 04.06.2023

Paraneoplastic retinopathy

Introduction

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.

• The pattern of vision loss and degree of retinal dysfunction can vary depending on the type of tumor, and a 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 to 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 (121). 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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