Table 1.
Functional pancreatic endocrine tumor (PET) syndromes
| Name | Biologically active peptide(s) secreted | Incidence (new cases/106 population/year) | Tumor location | Malignant % | Associated with MEN-1, % | Main symptoms/signs |
|---|---|---|---|---|---|---|
| A. Most common functional P Insulinoma | ET syndromes insulin | 1–3 | pancreas (>99%) | <10 | 4–5 | hypoglycemic symptoms (100%) |
| Zollinger-Ellison syndrome | gastrin | 0.5–2 | duodenum (70%); pancreas (25%); other sites (5%) | 60–90 | 20–25 | pain (79–100%); diarrhea (30–75%); esophageal symptoms (31–56%) |
| B. Established rare functional VIPoma (Verner-Morrison syndrome, pancreatic cholera, WDHA) | PET syndromes (RFT vasoactive intestinal peptide | s) 0.05–0.2 | pancreas (90%, adult); other (10%, neural, adrenal, periganglionic) | 40–70 | 6 | diarrhea (90–100%); hypokalemic (80–100%); dehydration (83%) |
| Glucagonoma | glucagon | 0.01–0.1 | pancreas (100%) | 50–80 | 1–20 | rash (67–90%); glucose intolerance (38–87%); weight loss (66–96%) |
| Somatostatinoma | somatostatin | rare | pancreas (55%); duodenum/jejunum (44%) | >70 | 45 | diabetes mellitus (63–90%); cholelithiases (65–90%); diarrhea (35–90%) |
| GRHoma | growth hormone-releasing hormone | unknown | pancreas (30%); lung (54%); jejunum (7%); other (13%) | >60 | 16 | acromegaly (100%) |
| ACTHoma | ACTH | rare | pancreas (4–16% all ectopic Cushing's) | >95 | rare | Cushing's syndrome (100%) |
| PET causing carcinoid syndrome | serotonin? tachykinins | rare (43 cases) | pancreas (<1% all carcinoids) | 60–88 | rare | same as carcinoid syndrome above |
| PET causing hypercalcemia (PTHrp-oma) | PTHrp; others unknown | rare | pancreas (rare cause of hypercalcemia) | 84 | rare | abdominal pain due to hepatic metastases, symptoms due to hypercalcemia |
| Possible rare functional PET s PET secreting calcitonin | yndromes calcitonin | rare | pancreas (rare cause of hypercalcitonemia) | >80 | 16 | diarrhea (50%) |
| PET secreting renin | renin | rare | pancreas | unknown | no | hypertension |
| PET secreting luteinizing hormone | luteinizing hormone | rare | pancreas | unknown | no | anovulation, virilization (female): reduced libido (male) |
| PET secreting erythropoietin | erythropoietin | rare | pancreas | 100 | no | polycythemia |
| PET secreting IF-II | insulin-like growth factor II | rare | pancreas | unknown | no | hypoglycemia |