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SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures

We describe an unusual case of a 55-year old female with a proinsulin secreting pancreatic endocrine adenoma. She presented with partial seizures, fasting hypoglycemia, and hypoglycemia unawareness. CT and MRI confirmed a hyper-enhancing lesion in the pancreatic body. She was effectively treated wit...

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Autores principales: Lyerla, Ryan, Shrestha, Rupendra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552798/
http://dx.doi.org/10.1210/js.2019-SUN-316
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author Lyerla, Ryan
Shrestha, Rupendra
author_facet Lyerla, Ryan
Shrestha, Rupendra
author_sort Lyerla, Ryan
collection PubMed
description We describe an unusual case of a 55-year old female with a proinsulin secreting pancreatic endocrine adenoma. She presented with partial seizures, fasting hypoglycemia, and hypoglycemia unawareness. CT and MRI confirmed a hyper-enhancing lesion in the pancreatic body. She was effectively treated with prednisone and utilized a continuous glucose monitor (CGM) to identify and treat hypoglycemia while she awaited surgery. She underwent resection and the surgical specimen was neuroendocrine adenomatous tissue. This case has several important teaching points: [1] Fasting hypoglycemia requires a complete work up since proinsulinomas, although rare, can occur. [2] Whipple’s triad is not 100% sensitive in the work-up of hypoglycemia. Patients with persistent hypoglycemia may develop hypoglycemia unawareness. [3] Continuous glucose monitors (CGM) may provide an alternative or adjunct to standard medical therapy while patients await definitive surgery. Prednisone may also be an inexpensive and effective therapy. References1. Fadini GP MAVAea. Hypoglycemic Syndrome in a Patient with Proinsulin-Only Secreting Pancreatic Adenoma (Proinsulinoma). Case Reports in Medicine. 2011.2. Clark G, Sadur C, Puligandla B. Multiple proinsulin-secreting tumors of the pancreas treated by laparoscopic distal pancreatectomy and splenectomy. World Journal of Surgery. 2009;33(3):397-3993. F. J. Gomez-Perez DCRPAVCAASRMaJAR. Beta-cell adenomas without hyperinsulinemia with use of highly specific insulin radioimmunoassays: case report and review of literature. Endocrine Practice. 2010;16(4):660-663.4. Gury H, Rio F, Neamtu D, Boivin S. Insulinoma with hyperproinsulinemia: A two cases report. Annales d'Endocrinologie. 2002;63(3):240-242.5. Piovesan A, Pia A, Visconti G, al e. Proinsulin-secreting neuroendocrine tumor of the pancreas. Journal of Endocrinological Investigation. 2003;26(8):758-761.
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spelling pubmed-65527982019-06-13 SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures Lyerla, Ryan Shrestha, Rupendra J Endocr Soc Tumor Biology We describe an unusual case of a 55-year old female with a proinsulin secreting pancreatic endocrine adenoma. She presented with partial seizures, fasting hypoglycemia, and hypoglycemia unawareness. CT and MRI confirmed a hyper-enhancing lesion in the pancreatic body. She was effectively treated with prednisone and utilized a continuous glucose monitor (CGM) to identify and treat hypoglycemia while she awaited surgery. She underwent resection and the surgical specimen was neuroendocrine adenomatous tissue. This case has several important teaching points: [1] Fasting hypoglycemia requires a complete work up since proinsulinomas, although rare, can occur. [2] Whipple’s triad is not 100% sensitive in the work-up of hypoglycemia. Patients with persistent hypoglycemia may develop hypoglycemia unawareness. [3] Continuous glucose monitors (CGM) may provide an alternative or adjunct to standard medical therapy while patients await definitive surgery. Prednisone may also be an inexpensive and effective therapy. References1. Fadini GP MAVAea. Hypoglycemic Syndrome in a Patient with Proinsulin-Only Secreting Pancreatic Adenoma (Proinsulinoma). Case Reports in Medicine. 2011.2. Clark G, Sadur C, Puligandla B. Multiple proinsulin-secreting tumors of the pancreas treated by laparoscopic distal pancreatectomy and splenectomy. World Journal of Surgery. 2009;33(3):397-3993. F. J. Gomez-Perez DCRPAVCAASRMaJAR. Beta-cell adenomas without hyperinsulinemia with use of highly specific insulin radioimmunoassays: case report and review of literature. Endocrine Practice. 2010;16(4):660-663.4. Gury H, Rio F, Neamtu D, Boivin S. Insulinoma with hyperproinsulinemia: A two cases report. Annales d'Endocrinologie. 2002;63(3):240-242.5. Piovesan A, Pia A, Visconti G, al e. Proinsulin-secreting neuroendocrine tumor of the pancreas. Journal of Endocrinological Investigation. 2003;26(8):758-761. Endocrine Society 2019-04-30 /pmc/articles/PMC6552798/ http://dx.doi.org/10.1210/js.2019-SUN-316 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Tumor Biology
Lyerla, Ryan
Shrestha, Rupendra
SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title_full SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title_fullStr SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title_full_unstemmed SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title_short SUN-316 Rare Neuroendocrine Tumor Presenting with Nocturnal Seizures
title_sort sun-316 rare neuroendocrine tumor presenting with nocturnal seizures
topic Tumor Biology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552798/
http://dx.doi.org/10.1210/js.2019-SUN-316
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