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Published on: August 18, 2023
Relation between ocular paraneoplastic syndromes and Immune Checkpoint Inhibitors (ICI): review of literature
Pauline Casselman1, Julie Jacob1, Pieter-Paul Schauwvlieghe2
1Department of Ophthalmology, University Hospitals Leuven, Herestraat 49, 3000, Leuven, Belgium.
Purpose:
To describe different ocular paraneoplastic syndromes in patients treated with Immune Checkpoint Inhibitors (ICI), its relation with different types of ICI and different types of tumors, and its implications for treatment.
Methods:
A comprehensive review of the literature was performed.
Results:
Patients treated with ICI can present with different ocular paraneoplastic syndromes, such as Carcinoma Associated Retinopathy (CAR), Melanoma Associated Retinopathy (MAR) and paraneoplastic Acute Exudative Polymorphous Vitelliform Maculopathy (pAEPVM). In literature, the different types of paraneoplastic retinopathy are mostly related to different types of primary tumors, with MAR and pAEPVM seen in melanoma, and CAR in carcinoma. Visual prognosis is limited in MAR and CAR.
Conclusion:
Paraneoplastic disorders result from an antitumor immune response against a shared autoantigen between the tumor and ocular tissue. ICI enhance the antitumor immune response, which can lead to increased cross-reaction against ocular structures and unmasking of a predisposed paraneoplastic syndrome. Different types of primary tumors are related to different cross-reactive antibodies. Therefore, the different types of paraneoplastic syndromes are related to different types of primary tumors and are probably unrelated to the type of ICI. ICI-related paraneoplastic syndromes often lead to an ethical dilemma. Continuation of ICI treatment can lead to irreversible visual loss in MAR and CAR. In these cases overall survival must be weighed against quality of life. In pAEPVM however, the vitelliform lesions can disappear with tumor control, which may involve continuation of ICI.
Insights
Immune checkpoint inhibitors (ICI) can trigger ocular paraneoplastic syndromes like CAR, MAR, and pAEPVM, linked to specific tumors. Managing these syndromes involves balancing cancer treatment with vision preservation.
Area of Science:
- Ophthalmology
- Oncology
- Immunology
Background:
- Immune checkpoint inhibitors (ICIs) harness the immune system to fight cancer.
- Ocular paraneoplastic syndromes can arise as a side effect of cancer treatments.
- Understanding these syndromes is crucial for patient management.
Purpose of the Study:
- To review ocular paraneoplastic syndromes in patients treated with ICIs.
- To correlate syndromes with ICI types and tumor origins.
- To discuss treatment implications.
Main Methods:
- A comprehensive literature review was conducted.
Main Results:
- Patients on ICIs may develop Carcinoma Associated Retinopathy (CAR), Melanoma Associated Retinopathy (MAR), or paraneoplastic Acute Exudative Polymorphous Vitelliform Maculopathy (pAEPVM).
- MAR and pAEPVM are associated with melanoma; CAR is linked to carcinoma.
- Visual prognosis is often poor for MAR and CAR.
Conclusions:
- Ocular paraneoplastic syndromes stem from immune responses targeting shared antigens.
- ICIs can exacerbate these responses, leading to ocular side effects.
- Syndromes are linked to tumor type, not necessarily ICI type.
- Treatment decisions require balancing survival and quality of life, especially concerning visual function.

