Version July 2025
G: grade; MEN: multiple endocrine neoplasia; PNEC: pancreatic neuroendocrine carcinoma; PNET: pancreatic neuroendocrine tumor; VHL: von Hippel-Lindau.
* Familial PNETs associated with MEN or VHL disease are excluded from this algorithm. For management of PNEC, refer to UpToDate content on poorly differentiated gastroenteropancreatic neuroendocrine carcinoma.
¶ The decision to pursue debulking surgery is a complex one and needs to take into account tumor biology, the volume of metastatic disease, the severity of any local or systemic symptoms, and the response to systemic therapy.
Δ Refer to UpToDate content on systemic therapy for metastatic well-differentiated G1 and G2 PNET. Nonsurgical liver-directed therapies include ablation and hepatic transarterial embolization.
◊ Most patients with metastatic PNET G3 are treated with systemic therapy. A minority of those with limited, low-burden, liver-isolated metastasis may be candidates for surgical resection.
