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Islet Hyperplasia in Adults: Challenge to Preoperatively Diagnose Non-Insulinoma Pancreatogenic Hypoglycemia Syndrome

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Abstract

Background

Pancreatic hyperfunctional islet hyperplasia in adults has been more and more frequently described in the literature. Postprandial neuroglycopenia, a negative normal fasting test, negative pancreatic imaging results, and positive intra-arterial calcium stimulation of serum insulin are characteristic. In affected patients the term non-insulinoma pancreatogenic hypoglycemia syndrome (NIPHS) was proposed.

Materials and Methods/Patients

We also encountered fasting hypoglycemia in such patients and therefore evaluated clinical and biochemical data in patients with NIPHS (n = 11), patients with insulinoma (n = 70), and patients in whom hypoglycemia was ruled out (n = 70).

Results

Patients with NIPHS were younger (median age: 41 years; range: 18–66) and mostly non-obese (median body mass index/BMI: 22.2 kg/m2; range: 19–39) compared with patients with an insulinoma (median age: 50 years; median: BMI 26.1 kg/m2). During an oral glucose tolerance test (OGTT) followed by a standard fasting test, neuroglycopenia was observed postprandially with a mean minimal blood glucose level of 36 ± 9 mg/dl in 7 out of 11 patients. Spontaneous hypoglycemia during the fast was 38 ± 5 mg/dl in 8 out of 11 patients. The corresponding insulin levels were 9.2 ± 9.8 mU/l (OGTT) and 6.8 ± 5.4 mU/l (fasting), significantly lower than in patients with insulinoma (P < 0.001), but not different from patients without hypoglycemia (P = 0.05). After pancreatic resection 8 patients (73%) were cured with enduring euglycemia. Pathohistological islet abnormalities with hyperplasia, hypertrophy, and microadenomatosis were confirmed in all patients.

Conclusion

In patients with postprandial and/or fasting neuroglycopenia NIPHS may be suspected when insulin levels are low but inadequately suppressed and localization studies failed to show a distinct pancreatic tumor.

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Abbreviations

MEN-1::

Multiple endocrine neoplasia type 1;

NIPHS::

Non-insulinoma pancreatogenic hypoglycemia syndrome;

PHHI::

Persistent hyperinsulinemic hypoglycemia of infancy;

SUR1 gene::

Sulfonylurea receptor-1 gene;

Kir6.2 gene:

Potassium inward rectifier 6.2 gene

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Authors and Affiliations

  1. Insulinoma and GEP Tumor Center Neuss-Düsseldorf, Lukaskrankenhaus Neuss, Preussenstrasse 84, Neuss, 41464, Germany

    Achim Starke MD, Christiane Saddig MD, Cyrus Tschahargane MD & Peter Goretzki MD

  2. Department of Internal Medicine, University Hospital, Heinrich-Heine-University, Moorenstrasse 5, Düsseldorf, 40225, Germany

    Achim Starke MD

  3. Institute of Pathology, Lukaskrankenhaus, Preussenstrasse 84, Neuss, 41464, Germany

    Barbara Kirch MD & Cyrus Tschahargane MD

  4. Department of Visceral and Endocrine Surgery, Lukaskrankenhaus, Preussenstrasse 84, Neuss, 41464, Germany

    Peter Goretzki MD

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  1. Achim Starke MD
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  2. Christiane Saddig MD
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  5. Peter Goretzki MD
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Correspondence to Achim Starke MD.

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Starke, A., Saddig, C., Kirch, B. et al. Islet Hyperplasia in Adults: Challenge to Preoperatively Diagnose Non-Insulinoma Pancreatogenic Hypoglycemia Syndrome. World J. Surg. 30, 670–679 (2006). https://doi.org/10.1007/s00268-005-0543-6

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