1. Glucagonoma Syndrome: A Rare Paraneoplastic Disorder due to Neuroendocrine Tumor of the Pancreas.
- Author
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Yusuf MA, Mehmood S, Iftikhar J, Saqib M, Siddique MZ, and Imtiaz W
- Subjects
- Female, Humans, Octreotide therapeutic use, Pancreas pathology, Rare Diseases, Glucagonoma complications, Glucagonoma diagnosis, Glucagonoma surgery, Necrolytic Migratory Erythema diagnosis, Necrolytic Migratory Erythema etiology, Necrolytic Migratory Erythema pathology, Neuroendocrine Tumors complications, Neuroendocrine Tumors diagnosis, Neuroendocrine Tumors surgery, Pancreatic Neoplasms complications, Pancreatic Neoplasms diagnosis, Pancreatic Neoplasms pathology
- Abstract
Glucagonoma syndrome is an extremely rare paraneoplastic disorder. The key presenting feature is a rash (necrolytic migratory erythema) which can easily be misdiagnosed as a primary skin disorder. Moreover, 50 to 80 % of patients already have metastatic disease at diagnosis. We report a case of a 38-year female presenting with epigastric pain and a skin rash all over the body. Workup revealed a neuroendocrine tumor (NET) of the pancreas, for which she underwent resection, resulting in a complete cure. A follow-up MRI after 8 months showed a hyperintense and arterially enhancing nodular liver lesion which did not show any uptake on the octreotide scan. However, a subsequent biopsy revealed a recurrence of the tumor. This was a unique finding in our case where a highly sensitive octreotide scan failed to identify metastasis, emphasising the importance of biopsy in such cases. Key Words: Glucagonoma, Necrolytic migratory erythema, Alpha-cell adenom.
- Published
- 2022
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