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Glucagonoma Syndrome: A Rare Paraneoplastic Disorder due to Neuroendocrine Tumor of the Pancreas.
- Source :
-
Journal of the College of Physicians and Surgeons--Pakistan : JCPSP [J Coll Physicians Surg Pak] 2022 Aug; Vol. 32 (8), pp. S147-S149. - Publication Year :
- 2022
-
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.
- 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
Subjects
Details
- Language :
- English
- ISSN :
- 1681-7168
- Volume :
- 32
- Issue :
- 8
- Database :
- MEDLINE
- Journal :
- Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
- Publication Type :
- Report
- Accession number :
- 36210677
- Full Text :
- https://doi.org/10.29271/jcpsp.2022.Supp2.S147