Cancer-associated retinopathy in ampullary pancreatic cancer

Abstract

A 64-year-old woman presented with bilateral visual loss with shimmering photopsias as the only clinical manifestation of an occult pancreatic ampullary adenocarcinoma causing duct dilatation. Abnormal electroretinograms led to suspicion of cancer-associated retinopathy (CAR), and CT of the abdomen showed an underlying pancreatic malignancy, detected with subclinical liver function tests following diagnosis of CAR. Biopsy showed a T2N0M0 ampullary adenocarcinoma. The patient was managed with Whipple’s procedure and adjuvant chemotherapy and has made a good recovery with no progression of her retinopathy. To our knowledge, this is one of the first descriptions of CAR in the context of pancreatic malignancy. It is atypical in its asymmetric presentation and favourable patient outcome. CAR is an important diagnosis to make, as ocular manifestations can be the only indication of an occult malignancy, resulting in a swifter diagnosis and potentially life-saving early intervention.

Ocular Paraneoplastic Syndromes: A Critical Review of Diffuse Uveal Melanocytic Proliferation and Autoimmune Retinopathy

“…74 That said, reports on visual function usually found progressive deterioration in most patients despite treatment of primary malignancy supplemented with local and systemic corticosteroids. [75][76][77][78][79][80] This prompted explorations of other therapies including high-dose intravenous corticosteroids, corticosteroids plus immunomodulating agents, systemic azathioprine, systemic cyclosporine, systemic mycophenolate, plasmapheresis, intravenous immunoglobulins, and several different monoclonal antibodies (eg, alemtuzumab, rituximab, etc.). The results have been mixed in terms of shortterm outcomes.…”

Ocular Paraneoplastic Syndromes

“…This results in symptoms of cone dysfunction, namely, photosensitivity as well as photopsias (type of visual hallucinations seen as light flashes), prolonged glare, decreased best-corrected visual acuity (BCVA), color discrimination or disturbed color vision, and central scotomas. Rod dysfunction may include night blindness, prolonged adaptation to darkness, and peripheral or ring scotomas [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 ].…”

“…Central or ring scotomas should be detected in visual field examination. In electroretinography (ERG), rod- and cone-mediated responses are not recordable or significantly decreased, firstly affecting the a-wave and subsequently lining rapidly to a “flat ERG” [ 8 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ].…”