Consensus Recommendations for the Diagnosis and Management of Pancreatic Neuroendocrine Tumors: Guidelines from a Canadian National Expert Group

Abstract

Pancreatic neuroendocrine tumors (pNETs) are rare heterogeneous tumors that have been steadily increasing in both incidence and prevalence during the past few decades. Pancreatic NETs are categorized as functional (F) or nonfunctional (NF) based on their ability to secrete hormones that elicit clinically relevant symptoms. Specialized diagnostic tests are required for diagnosis. Treatment options are diverse and include surgical resection, intraarterial hepatic therapy, and peptide receptor radionuclide therapy (PRRT). Systemic therapy options include targeted agents as well as chemotherapy when indicated. Diagnosis and management should occur through a collaborative team of health care practitioners well-experienced in managing pNETs. Recent advances in pNET treatment options have led to the development of the Canadian consensus document described in this report. The discussion includes the epidemiology, classification, pathology, clinical presentation and prognosis, imaging and laboratory testing, medical and surgical management, and recommended treatment algorithms for pancreatic neuroendocrine cancers.

Neutrophil-lymphocyte ratio predicts survival in pancreatic neuroendocrine tumors

“…Unlike functioning neoplasms, non-functioning PNETs are often detected at an advanced stage due to the lack of specific symptoms (6). PNETs are highly heterogeneous neoplasms presenting a spectrum of biologic behavior (7,8). Aggressive progression can even be observed in incidentally detected and small tumors (9).…”

Principles of diagnosis and management of neuroendocrine tumours

“…The Canadian National Expert Group on NETs recently endorsed the staging groupings described in the 7th edition of the American Joint Committee on Cancer Staging Manual. 7,8,21 However, for practical purposes, NETs can be considered as either early stage (completely resectable) or advanced stage (either locally advanced and unresectable or metastatic).…”

“…38 Therefore, patients should be given a somatostatin analogue before any anesthetic or tumour manipulation. 7,8 How are neuroendocrine tumours diagnosed?…”

“…Canadian consensus guidelines recommend that all patients with a suspected NET should receive CTs of the chest, abdomen and pelvis for staging, and consideration of an MRI of the liver or pancreas in cases where further definition is required. 7,8 The second modality is functional imaging, which takes advantage of the overexpression of somatostatin receptors commonly seen in NETs. Radiolabelled somatostatin analogues are administered intravenously, concentrate in NETs, and the emitted radiation is detected to localize tumours.…”

“…All recommendations are consistent with current Canadian national guidelines. 7,8 Box 1 summarizes the evidence supporting this review.…”