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A rare pancreatic neuroendocrine tumor with proinsulin-secreting activity

https://doi.org/10.14341/serg12342

Abstract

This paper presents a clinical case describing hypoglycemic condition with atypical symptoms in a young patient in the early postpartum period. Abdominal ultrasound revealed a large formation in the hook region of the pancreas, which was subsequently confirmed according to endoscopic ultrasound, CT and MRI. The idea of insulinoma was formed according to the presence of pancreatic formation, documented Whipple’s triad, and data on hyperinsulinism at the outpatient stage. However, the 72-hour fasting test showed no significant increase in insulin and C-peptide levels. After excluding other causes of hypoglycemia, the patient was suspected of having proinsulinoma, which was confirmed by increased blood proinsulin level. Gastropancreatoduodenal resection was performed. We detected a highly differentiated neuroendocrine pancreatic tumor with the expression of CD56, NSE, synaptophysin and chromogranin A, with a Ki-67 index of about 1%. After surgical treatment, hypoglycemic conditions were not observed.

About the Authors

Anna R. Volkova
First Pavlov State Medical University of Saint Petersburg
Russian Federation

MD, PhD, Professor



Svetlana V. Dora
First Pavlov State Medical University of Saint Petersburg
Russian Federation

MD, PhD



Elena N. Ostrouchova
First Pavlov State Medical University of Saint Petersburg
Russian Federation

MD, PhD



Anna V. Lisker
First Pavlov State Medical University of Saint Petersburg
Russian Federation

MD



Galina V. Semikova
First Pavlov State Medical University of Saint Petersburg
Russian Federation

MD



Ekateryna U. Demydenko
First Pavlov State Medical University of Saint Petersburg
Russian Federation

medical resident



References

1. Beltsevich DG. ENETS consensus guidelines for the management of patients with digestive neuroendocrine neoplasms of the digestive system. Endocrine Surgery. 2012;6(3):8–40. (In Russ.). doi: https://doi.org/10.14341/2306-3513-2012-3-8-40

2. Murtha TD, Lupsa BC, Majumdar S, et al. A Systematic Review of Proinsulin-Secreting Pancreatic Neuroendocrine Tumors. J Gastrointest Surg. 2017;21(8):1335-1341. doi: https://doi.org/10.1007/s11605-017-3428-8

3. de Herder WW, Niederle B, Scoazec J-Y, et al. Well-Differentiated Pancreatic Tumor/Carcinoma: Insulinoma. Neuroendocrinology. 2006;84(3):183-188. doi: https://doi.org/10.1159/000098010

4. Piovesan A, Pia A, Visconti G, et al. Proinsulin-secreting neuroendocrine tumor of the pancreas. J Endocrinol Invest. 2003;26(8):758-761. doi: https://doi.org/10.1007/BF03347360

5. Gama R, Marks V, Wrlght J, Teale JD. Octreotide exacerbated fasting hypoglycaemia in a patient with a proinsulinoma; the glucostatic importance of pancreatic glucagon. Clin Endocrinol (Oxf ). 1995;43(1):117-120. doi: https://doi.org/10.1111/j.1365-2265.1995.tb01901.x

6. Cryer PE, Axelrod L, Grossman AB, et al. Evaluation and Management of Adult Hypoglycemic Disorders: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2009;94(3):709-728. doi: https://doi.org/10.1210/jc.2008-1410

7. Yukina MY, Nuralieva NF, Troshina EA, et al. The hypoglycemic syndrome (insulinoma): pathogenesis, etiology, laboratory diagnosis (review, part 1). Problems of Endocrinology. 2017;63(4):245–256. (In Russ.). doi: https://doi.org/10.14341/probl2017634245-256

8. Volkova NI, Porksheyan MI, Kanayeva MA, et al. Clinical case of rare neuroendocrine tumor of pancreas — proinsulinoma. Endocrine surgery. 2015;9(4):26–31. (In Russ.). doi: https://doi.org/10.14341/serg2015426-31.


Supplementary files

1. Figure 1. Ultrasound examination of the abdominal organs with formation in the pancreas.
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Type Исследовательские инструменты
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2. Figure 2. MRI of the abdominal organs with contrast enhancement. Arrows show the formation in the pancreas.
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Review

For citations:


Volkova A.R., Dora S.V., Ostrouchova E.N., Lisker A.V., Semikova G.V., Demydenko E.U. A rare pancreatic neuroendocrine tumor with proinsulin-secreting activity. Endocrine Surgery. 2020;14(2):16-20. (In Russ.) https://doi.org/10.14341/serg12342

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ISSN 2306-3513 (Print)
ISSN 2310-3965 (Online)