Regression of Paraneoplastic Optic Neuropathy Associated with Glucagonoma After Surgical Resection

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Introduction: Glucagonomas-associated ocular manifestations have been exceptionally reported.

Aim(s): To report the case of a patient with a glucagonoma associated with paraneoplastic optic neuropathy (PON) and to review literature.

Materials and methods: A 65-year-old man presented with bilateral visual loss and central scotoma. Later, the occurrence of diarrhoea, necrolytic migratory erythema evocated a glucagonoma syndrome, which was advocated by elevated chromogranin A and glucagon levels. CT scan showed a pancreatic tumor, which was confirmed to be a well-differentiated neuroendocrine tumor at pathological examination of biopsies. Surgical resection was performed and led to the dramatic improvement of bilateral visual acuity, and complete resolution of scotomas. Hence, the retained diagnosis was a pancreatic glucagonoma associated with a PON.

Conference: 12th Annual ENETSConcerence (2015)

Presenting Author:

Authors: Defour L, De Mestier L, Diebold M, Arndt C, Kianmanesh R,

Keywords: Paraneoplastic syndrome, glucagonoma, optic neuropathy.,