Jornal do Pâncreas Acesso livre

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Regression of a Glucagonoma-Related Paraneoplastic Optic Neuropathy after Surgical Resection

Lucie Defour, Louis de Mestier, Marie-Danièle Diebold, Carl Arndt, Reza Kianmanesh, Guillaume Cadiot

Introduction Glucagonoma-associated ophthalmological manifestations have been exceptionally reported. The purpose was to report the case of a patient with a glucagonoma-related paraneoplastic optic neuropathy that regressed after tumour resection, and provide a comprehensive literature review. Case report A Sixty-five-year-old man presented with bilateral visual loss and central scotoma. The occurrence of diarrhoea and necrolytic migratory erythema were suggestive of a glucagonoma syndrome, which was confirmed by elevated glucagon levels. Computed tomography scan showed a pancreatic tumour, which was a well-differentiated neuroendocrine tumour at pathological examination. Surgical resection was performed and led to the improvement of bilateral visual acuity and complete resolution of scotomas. Discussion The association of paraneoplastic optic neuropathy with pancreatic neuroendocrine tumours have been described 6 and 4 times in the literature, respectively. In larger series, glucagonoma-associated ophthalmological manifestations were reported in up to 14% of cases. Conclusion Glucagonomas may be associated with paraneoplastic optic neuropathy, which can regress after tumour resection.