Ocular Paraneoplastic Syndromes

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.

Glaucoma uveítico como síndrome paraneoplásico asociado a cáncer de mama

“…Parece que el control de la neoplasia primaria no influye en la evolución del proceso visual. Además de la retinopatía asociada al cáncer se han descrito otros síndromes paraneoplásicos, como la proliferación melanocítica uveal difusa y la neuropatía óptica paraneoplásica 12 .…”

Bilateral Serpiginous-Like Chorioretinitis Associated With Ciliochoroidal Melanoma

“…Ocular paraneoplastic syndromes result mainly from immunopathological factors including autoimmune and ectopic peptides processes. 7 Because autoimmune processes are major factors in the pathogenesis of both SC and ocular paraneoplastic syndrome, 1,2 it is reasonable to postulate that SLC can develop as a paraneoplastic condition. In fact, three previous reports described an association between SC and systemic malignancies, including lung carcinoma, 4 uterine cervix carcinoma, 5 and non-Hodgkin lymphoma.…”

Retrobulbar Optic Neuritis Induced by Pembrolizumab in a Patient with Lung Adenocarcinoma

“…Anti-Hu antibody is useful for diagnosing paraneoplastic neurological syndrome (specificity of 99% and sensitivity of 82%) ( 8 ). Paraneoplastic optic neuropathies are rarer than other retinopathies ( 9 ). In this case, paraneoplastic optic neuropathy cannot be excluded, but the anti-Hu antibody was negative.…”