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J Coll Physicians Surg Pak · Aug 2022
Case ReportsGlucagonoma Syndrome: A Rare Paraneoplastic Disorder due to Neuroendocrine Tumor of the Pancreas.
- Muhammed Aasim Yusuf, Shafqat Mehmood, Jhanzeb Iftikhar, Muhammad Saqib, Muhammad Zeeshan Siddique, and Wania Imtiaz.
- Department of Internal Medicine, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, Pakistan.
- J Coll Physicians Surg Pak. 2022 Aug 1; 32 (8): S147-S149.
AbstractGlucagonoma 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.
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