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Insulinoma: only in adults?—case reports and literature review

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Abstract

Insulinomas first presenting as refractory seizure disorders are well documented in adulthood but rarely found in children. Only a few cases of childhood insulinoma have been reported so far. We report on two adolescents with hyperinsulinaemic hypoglycaemia, initially misdiagnosed as epilepsy and migraine accompagnée, and compare those to other cases published. Localization of insulinoma was challenging and, in one patient, angiography with selective arterial calcium stimulation and hepatic venous sampling in addition to CT and MRI was necessary. In these patients, long-term recovery was achieved by laparoscopic distal pancreatic resection in one and by conventional enucleation in the pancreatic head in the second patient. In contrast to adults, macrosomy and a decrease in school performance were the main symptoms and, during fasting, impaired cognitive function occurred after a relatively short period and at a higher glucose threshold or lower insulin/glucose ratio, respectively. Neuroglycopenic signs may be attributed to behaviour abnormalities or seizure disorders but in children and adolescents may already be caused by insulinoma. In these cases, timely diagnosis as well as tumour resection ensure long-term cure.

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Notes

  1. Thanks to Peter Waibel, MD, Ostschweiter Kinderspital, St. Gallen, Switzerland.

  2. Histological examination kindly performed by Ch. Öhlschlegel, MD, Institute for Pathology, Kantonsspital St. Gallen, Switzerland.

Abbreviations

ASVS:

Selective arterial calcium stimulation and hepatic venous sampling

CHI:

Congenital hyperinsulinism

CT:

Computed tomography

[18F]-DOPA PET/CT scan:

Fluorine-18 l-3,4-dihydroxyphenylalanine positron emission tomography/computed tomography scan

HH:

Hyperinsulinaemic hypoglycaemia

MCAD:

Medium-chain acyl-coenzyme A dehydrogenase deficiency

MEN::

Multiple endocrine neoplasia

MMSE:

Mini-mental state examination

MRI:

Magnetic resonance imaging

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Conflict of interest

The authors declare that they have no conflict of interest.

Funding

This research did not receive any specific grant from any funding agency in the public, commercial or non-profit sector.

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Authors and Affiliations

  1. Clinic of Adolescent Medicine, Division of Paediatric Endocrinology and Diabetology, Ostschweizer Kinderspital, Claudiusstrasse 6, 9006, St. Gallen, Switzerland

    Tiziana Gozzi Graf & Dagmar l’Allemand

  2. Division of Endocrinology, Diabetes and Osteology, Department of Internal Medicine, Kantonsspital, St. Gallen, Switzerland

    Michael Brändle

  3. Department of Surgery, Kantonsspital, St. Gallen, Switzerland

    Thomas Clerici

  4. Department of Paediatrics, Kantonsspital Luzern, Lucerne, Switzerland

    Dagmar l’Allemand

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  1. Tiziana Gozzi Graf
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  2. Michael Brändle
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  4. Dagmar l’Allemand
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Correspondence to Tiziana Gozzi Graf.

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Gozzi Graf, T., Brändle, M., Clerici, T. et al. Insulinoma: only in adults?—case reports and literature review. Eur J Pediatr 173, 567–574 (2014). https://doi.org/10.1007/s00431-013-2005-8

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  • DOI: https://doi.org/10.1007/s00431-013-2005-8

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