World Journal of Surgery

, Volume 30, Issue 5, pp 670–679 | Cite as

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

  • Achim Starke
  • Christiane Saddig
  • Barbara Kirch
  • Cyrus Tschahargane
  • Peter Goretzki



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).


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.


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.



Multiple endocrine neoplasia type 1;


Non-insulinoma pancreatogenic hypoglycemia syndrome;


Persistent hyperinsulinemic hypoglycemia of infancy;

SUR1 gene:

Sulfonylurea receptor-1 gene;

Kir6.2 gene

Potassium inward rectifier 6.2 gene


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Copyright information

© Société Internationale de Chirurgie 2006

Authors and Affiliations

  • Achim Starke
    • 1
    • 2
  • Christiane Saddig
    • 1
  • Barbara Kirch
    • 3
  • Cyrus Tschahargane
    • 1
    • 3
  • Peter Goretzki
    • 1
    • 4
  1. 1.Insulinoma and GEP Tumor Center Neuss-DüsseldorfLukaskrankenhaus NeussNeussGermany
  2. 2.Department of Internal MedicineUniversity Hospital, Heinrich-Heine-UniversityDüsseldorfGermany
  3. 3.Institute of PathologyLukaskrankenhausNeussGermany
  4. 4.Department of Visceral and Endocrine SurgeryLukaskrankenhausNeussGermany

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