Table 1.
Characteristics of PanNETs.
PanNET Type | Associated Biomarkers/Hormones | Associated Genetic Syndromes | Clinical Manifestations | Diagnosis | Imaging |
---|---|---|---|---|---|
Insulinoma | Insulin Pro-insulin C-Peptide Chromogranin A |
MEN1 | Hypoglycemia | 72 h fasting test Negative serum/urine toxicology for insulin secretagogues |
Ultrasound/CT/MRI/EUS Somatostatin receptor scintigraphy (octreotide scan) Arteriography/intraarterial stimulation with venous sampling 18-F-DOPA PET 68Ga-DOTATATE PET/CT |
Gastrinoma | Gastrin Chromogranin A |
MEN1 | Zollinger–Ellison syndrome | Gastrin levels Stomach pH Secretin test |
EUS Somatostatin receptor scintigraphy (octreotide scan) 68Ga-DOTATATE PET/CT |
Glucagonoma | Glucagon Chromogranin A Neuron-specific enolase |
MEN1 | Migratory necrolytic erythema | Glucagon levels | Ultrasound/CT Somatostatin receptor scintigraphy (octreotide scan) |
VIPoma | Vasoactive intestinal polypeptide Gastrin Insulin |
MEN1 | Secretory diarrhea | VIP levels Stool osmolality 68Ga-DOTATATE PET/CT |
68Ga-DOTATATE PET/CT |
Somatostatinoma | Somatostatin | MEN1 | Somatostatinoma syndrome | EUS-FNA | Ultrasound/CT/MRI |
Nonfunctional PanNETs | Chromogranin A Polypeptide P |
VHL | EUS |