Follow-up and quality of life in patients with non-single-insulinoma pancreatogenic hypoglycemia syndrome (NIPHS) – 11 years of experience in one center

Purpose: Insulinoma and non-single-insulinoma pancreatogenic hypoglycemia syndrome (NIPHS) are rare causes of hypoglycemia. Treatment options and postoperative outcome are discussed to define future strategies.

Methods: We retrospectively analyzed all patients with neuroendocrine tumours (NEN) treated between 2001 and 2012. Data related to diagnosis (oral-glucose-tolerance-test, selective arterial calcium-injection test, imaging) and therapy were evaluated. Postoperative outcome (diabetes mellitus, relapse of hypoglycaemia) and quality of life (QoL) was assessed by standardized questionnaires and/or phone interview.

Results: Of 202 patients with NEN 61 (31%) demonstrated with insulinoma, 17 (8%) suffered from NIPHS. None of these had undergone bariatric surgery. The 14 female and 3 male patients showed a median age of 43 years (range 24 – 70). 14 of 17 patients with NIPHS (82%) were operated on (left/extended left resection n = 11, modified Whipple's procedure n = 2, uncinate plus left resection n = 1). 5 patients (36%) suffered from recurrent disease 11 months (range 5 – 36) after surgery, followed by re-operation in 3 (21%). One patient (7%) had a second recurrence. 3 of 14 patients developed Diabetes mellitus (21%). The overall QoL improved in 9 (64%), and worsened in 4 patients (29%) (unknown in one patient, who died from myocardial infarction). Three patients without surgery were on diet without problems. Median follow-up intervall was 53 month (range 6 – 121).

Conclusions: Majority of patients with NIPHS demonstrate relief from symptoms and improved quality of life after 80% pancreatectomy, however recurrence often develop after surgery.