The Landmark Series: Surgical Management of Functioning and Non-Functioning Pancreatic Neuroendocrine Tumors

Abstract

Pancreatic neuroendocrine tumors (PNETs) are comparatively rare pancreatic malignancies that exhibit diverse biologic behavior, ranging from indolent tumors to widely metastatic cancers, with up to 15 % secreting hormones that cause symptoms. As a consequence, the management of PNETs is highly individualized and can include active surveillance of small (1-2 cm) and very small (< 1 cm) nonfunctioning tumors without worrisome features, parenchymal-sparing resection of appropriately located tumors, anatomic pancreatectomy and, in select cases, debulking of metastatic disease, particularly in the liver. This review synthesizes society recommendations and contemporary evidence guiding the surgical management of PNETs. Innovations in molecular profiling and systemic therapies hold promise to refine surgical algorithms for this heterogeneous tumor.

Keywords: Active surveillance; Liver debulking; Minimally invasive surgery; Pancreatic neuroendocrine neoplasm; Pancreatic neuroendocrine tumor.

Fig. 1

Computed tomography (CT) of the abdomen with intravenous (IV) contrast of a ~ 1 cm non-functioning PNET in the uncinate process (orange arrows) under active surveillance and without evidence of locoregional or distant metastases on DOTATATE PET CT (green arrows). PNET, pancreatic neuroendocrine tumor; PET, positron emission tomography

Fig. 2

Magnetic resonance imaging (MRI) of the abdomen showing A a PNET in the tail of the pancreas (T2 sequence, green arrow) and B liver metastases (EOVIST phase, orange arrows). C surgical specimen and D liver after primary tumor resection and liver-debulking using parenchymal-sparing techniques and ultrasound-guided microwave ablation