Successful Medical Treatment of Adult Nesidioblastosis With Pasireotide over 3 Years: A Case Report.

Schwetz, Verena; Horvath, Karl; Kump, Patrizia; Lackner, Carolin; Perren, Aurel; Forrer, Flavio; Pieber, Thomas R; Treiber, Gerlies; Sourij, Harald; Mader, Julia K (2016). Successful Medical Treatment of Adult Nesidioblastosis With Pasireotide over 3 Years: A Case Report. Medicine, 95(14), e3272. Lippincott Williams & Wilkins 10.1097/MD.0000000000003272

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Nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Diagnosis is often challenging and therapeutic options are scarce.In 2009, a 46-year-old female patient presented with recurrent severe hypoglycemia and immediate recovery after glucose ingestion. Although 72-h-fasting test was positive, various imaging technologies (sonography, computed tomography, somatostatin receptor scintigraphy, dopamine receptor positron emission tomography [DOPA-PET]) were negative. Endoscopic ultrasound revealed a lesion in the pancreatic corpus, whereas selective arterial calcium stimulation test, portal venous sampling and GLP-1-receptor scintigraphy were indicative of a lesion in the pancreatic tail, which was surgically removed. The histopathologic examination revealed beta cell hyperplasia and microadenomas expressing glucagon. After surgery, the patient was free of symptoms for 6 months, after which hypoglycemic episodes recurred. After unsuccessful treatment with corticosteroids and somatostatin analogs, treatment with pasireotide, a novel somatostatin analog with high affinity to somatostatin receptor 5 and a possible side effect of hyperglycemia, was initiated (0.6 mg BID). To date, our patient has been free of severe hypoglycemic episodes ever since. Yearly repeated imaging procedures have shown no abnormities over the last 3 years.We report for the first time that pasireotide was successfully used in the treatment of adult nesidioblastosis.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Tumour Pathology

UniBE Contributor:

Perren, Aurel

Subjects:

500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health

ISSN:

0025-7974

Publisher:

Lippincott Williams & Wilkins

Language:

English

Submitter:

Aurel Perren

Date Deposited:

22 Dec 2016 08:37

Last Modified:

05 Dec 2022 15:00

Publisher DOI:

10.1097/MD.0000000000003272

PubMed ID:

27057885

BORIS DOI:

10.7892/boris.91976

URI:

https://boris.unibe.ch/id/eprint/91976

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