Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Nov 10;8(11):490.
doi: 10.3390/biomedicines8110490.

Ocular Paraneoplastic Syndromes

Joanna Przeździecka-Dołyk  1   2 , Anna Brzecka  3 , Maria Ejma  4 , Marta Misiuk-Hojło  1 , Luis Fernando Torres Solis  5 , Arturo Solís Herrera  6 , Siva G Somasundaram  7 , Cecil E Kirkland  7 , Gjumrakch Aliev  8   9   10   11
Affiliations
Review

Ocular Paraneoplastic Syndromes

Joanna Przeździecka-Dołyk et al. Biomedicines. .

Abstract

Ocular-involving paraneoplastic syndromes present a wide variety of clinical symptoms. Understanding the background pathophysiological and immunopathological factors can help make a more refined differential diagnosis consistent with the signs and symptoms presented by patients. There are two main pathophysiology arms: (1) autoimmune pathomechanism, which is presented with cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), cancer-associated cone dysfunction (CACD), paraneoplastic vitelliform maculopathy (PVM), and paraneoplastic optic neuritis (PON), and (2) ectopic peptides, which is often caused by tumor-expressed growth factors (T-exGF) and presented with bilateral diffuse uveal melanocytic proliferation (BDUMP). Meticulous systematic analysis of patient symptoms is a critical diagnostic step, complemented by multimodal imaging, which includes fundus photography, optical coherent tomography, fundus autofluorescence, fundus fluorescein angiography, electrophysiological examination, and sometimes fundus indocyjanin green angiography if prescribed by the clinician. Assessment of the presence of circulating antibodies is required for diagnosis. Antiretinal autoantibodies are highly associated with visual paraneoplastic syndromes and may guide diagnosis by classifying clinical manifestations in addition to monitoring treatment.

Keywords: cancer-associated cone dysfunction; cancer-associated retinopathy; extracellular vesicles; melanoma-associated retinopathy; optic neuritis; paraneoplastic syndromes; uveal melanocytic proliferation; vitelliform maculopathy.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
The pathophysiology of ocular paraneoplastic syndromes (OPNS). Autoimmune pathomechanism is presented with cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), cancer-associated cone dysfunction (CACD), paraneoplastic vitelliform maculopathy PVM), and paraneoplastic optic neuritis (PON). Ectopic peptides, caused by tumor-expressed growth factors (T-exGF), are presented with bilateral diffuse uveal melanocytic proliferation (BDUMP) and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (POEMS).
Figure 2
Figure 2
Mechanism of retinal invasion of autoantibodies and lymphocytes during CAR.
See this image and copyright information in PMC

References

    1. Rahimy E., Sarraf D. Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management. Surv. Ophthalmol. 2013;58:430–458. doi: 10.1016/j.survophthal.2012.09.001. - DOI - PubMed
    1. Darnell R.B., Posner J.B. Paraneoplastic syndromes involving the nervous system. N. Engl. J. Med. 2003;349:1543–1554. doi: 10.1056/NEJMra023009. - DOI - PubMed
    1. De Salvo G., Prakash P., Rennie C.A., Lotery A.J. Long-term survival in a case of bilateral diffuse uveal melanocytic proliferation. Eye. 2011;25:1385–1386. doi: 10.1038/eye.2011.180. - DOI - PMC - PubMed
    1. Adamus G. Latest updates on antiretinal autoantibodies associated with vision loss and breast cancer. Investig. Ophthalmol. Vis. Sci. 2015;56:1680–1688. doi: 10.1167/iovs.14-15739. - DOI - PMC - PubMed
    1. Dalin F., Adamus G., Yang S., Landgren E., Palle J., Hallgren Å., Frost B.-M., Hugosson T., Landegren N., Eriksson D., et al. Aryl Hydrocarbon Receptor-Interacting Protein-Like 1 in Cancer-Associated Retinopathy. Ophthalmology. 2016;123:1401–1404. doi: 10.1016/j.ophtha.2015.12.031. - DOI - PubMed

LinkOut - more resources