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Case Reports
. 2024 Sep 7:36:102166.
doi: 10.1016/j.ajoc.2024.102166. eCollection 2024 Dec.

An unusual pAIR: Anti-PKM2 antibody and occult pancreatic adenocarcinoma

Meredith P Spitz  1 , David R Anderson  2 , Tamara R Vrabec  1
Affiliations
Case Reports

An unusual pAIR: Anti-PKM2 antibody and occult pancreatic adenocarcinoma

Meredith P Spitz et al. Am J Ophthalmol Case Rep. .

Abstract

Purpose: To describe the clinical, laboratory and multimodal imaging findings in paraneoplastic autoimmune retinopathy (p-AIR) associated with anti-pyruvate kinase M2 antibody (anti-PKM2) and occult pancreatic adenocarcinoma.

Observations: A 70 year old male with blurred vision, nyctalopia and concurrent difficulty with glucose control had retinal vascular attenuation and diffuse punctate pigment clumping in both eyes. Multimodal imaging demonstrated corresponding stippled hypofluorescence on fluorescein angiography, stippled hyperautofluorescence and a hyperautoflourescent macular ring with fundus autofluorescence, and focal hyperreflectivity at the level of the RPE-Bruch's membrane complex with diffuse loss of outer retinal layers on ocular coherence tomography. In addition, diffuse ganglion cell loss and severe visual field constriction were present. Genetic testing for retinitis pigmentosa was normal. Screening for anti-retinal antibodies was positive for only anti-PKM2. Systemic evaluation revealed previously undiagnosed adenocarcinoma of the pancreas.

Conclusions and importance: Anti-PKM2 in the setting of autoimmune retinopathy may be associated with occult pancreatic cancer. The diagnosis of pAIR should be considered and systemic investigation for occult malignancy initiated even in the absence of more commonly associated anti-retinal antibodies.

Keywords: Anti-pyruvate kinase M2 antibody; Cancer associated retinopathy; Pancreatic adenocarcinoma; Paraneoplastic autoimmune retinopathy; Pyruvate kinase M2; anti-retinal autoantibodies.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this manuscript.

Figures

Fig. 1
Fig. 1
Clinical appearance and multimodal imaging. Fundus photograph demonstrates attenuated retinal vasculature. Diffuse punctate hyperpigmentation is present throughout (1a, arrow). FAF shows hyperautofluorescence ring centered around the fovea and widespread punctate hyper-autofluorescence which corresponded with the clinically evident pigmented lesions (1b, arrow). Fluorescein angiography reveals coarsely stippled pattern of hypofluorescence which corresponds to the pigmented fundus on a hyperfluorescent background (1c, arrow). Attenuation of outer retinal layers with photoreceptor cell loss is evident on OCT OU. Line scan demonstrates deposits at the level of the RPE correlate with pigmented fundus lesions (1d, arrows).
Fig. 2
Fig. 2
Visual fields at presentation are remarkable for severe constriction in both eyes.
Fig. 3
Fig. 3
MRI demonstrates spiculated mass at the pancreatic head which encases the superior mesenteric vein and abuts the superior mesenteric artery (asterisk). Local lymphadenopathy is present.
Fig. 4
Fig. 4
Fundus autoflourescence at presentation (top) compared to 14 months later (bottom) demonstrates modest constriction and decrease in autoflourescence in both eyes.
See this image and copyright information in PMC

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