Pancreatic neuroendocrine tumors (PNETs) are hormone-secreting tumors of the pancreas that derive from neuroendocrine cells. The most important types are glucagonomas, VIPomas, somatostatinomas, insulinomas (treated in a separate article), and gastrinomas (treated in a separate article). With the exception of insulinomas, PNETs typically manifest with diarrhea, weight loss, and normal or elevated blood glucose levels; insulinomas do not affect bowel movements and manifest instead with weight gain and hypoglycemia. Diagnosis involves laboratory studies to identify excess hormone levels and imaging to detect the primary tumor and metastases. Surgical resection is the first-line treatment. Octreotide is the treatment of choice for inoperable tumors.
For more information, see “” and “ .”
|Overview of pancreatic neuroendocrine tumors|
|Affected cells||Blood glucose||Diarrhea||Other clinical features||Associated conditions|
|Glucagonoma|| || |
|Insulinoma|| || || |
|Gastrinoma|| || || |
|VIPoma|| || || |
|Somatostatinoma|| || |
- Definition: a rare neuroendocrine tumor of the pancreatic α-cells that secretes glucagon. In > 50% of cases, metastasis is present at diagnosis.
- Weight loss
- Impaired glucose tolerance or diabetes mellitus (75–95%)
Necrolytic migratory erythema
- A cutaneous paraneoplastic syndrome that is mainly associated with pancreatic tumors secreting glucagon, but also hepatitis B, C, and bronchial carcinoma
- Occurrence of multiple areas of centrifugally spreading erythema, located predominantly on the face, perineum, and lower extremities
- Develop into painful and pruritic crusty patches with central areas of bronze-colored induration
- Tend to resolve and reappear in a different location
- Skin biopsy shows epidermal necrosis
- Chronic diarrhea
- Diagnostics: requires a high index of suspicion to make the diagnosis
- Definition: a rare neuroendocrine tumor of δ-cell (D-cell) origin that is usually located in the pancreas or gastrointestinal tract and secretes somatostatin.
- Clinical features
- Definition: : a neuroendocrine tumor that secretes VIP (vasoactive intestinal polypeptide)
- Etiology: associated with (5% of cases)
- Tumor location: The primary tumor is most frequently found in the pancreas.
- Clinical features