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Surgical resection of sporadic pancreatic neuroendocrine tumors

James Lee, MD
John Allendorf, MD, FACS
John Chabot, MD
Section Editors
Sally E Carty, MD, FACS
Stanley W Ashley, MD
Deputy Editors
Wenliang Chen, MD, PhD
Diane MF Savarese, MD


Pancreatic neuroendocrine tumors (PNETs) are rare, accounting for less than 3 percent of all pancreatic tumors [1,2]. PNETs exhibit a wide spectrum of clinical behavior that has made classification and staging difficult. While the majority of PNETs are associated with relatively good survival, there can be significant variability in outcomes based on their biological heterogeneity [3-5]. The classification, epidemiology, clinical presentation, localization, and staging of PNETs is discussed separately. (See "Classification, epidemiology, clinical presentation, localization, and staging of pancreatic neuroendocrine tumors (islet-cell tumors)".)

The term "pancreatic neuroendocrine tumor" is preferred to encompass a diverse set of tumors arising in the pancreas that share a common progenitor cell [6]. In the past, PNETs were often referred to as pancreatic "islet cell" tumors or pancreatic "carcinoids". With time, the term carcinoid has come to mean a very specific group of tumors, predominantly arising in the tubular gastrointestinal tract, which produces only serotonin. However, endocrine tumors of the pancreas can express other hormones or can be biochemically inert. (See "Pathology, classification, and grading of neuroendocrine tumors arising in the digestive system", section on 'Historical evolution'.)

While most PNETs are sporadic, PNETs can also be associated with genetic syndromes. The surgical management of sporadic PNETs is reviewed here. The management of PNETs associated with genetic syndromes is discussed separately. (See "Multiple endocrine neoplasia type 1: Treatment" and "Clinical features, diagnosis, and management of von Hippel-Lindau disease" and "Tuberous sclerosis complex: Management and prognosis" and "Neurofibromatosis type 1 (NF1): Management and prognosis".)


Surgical planning must take into account the characteristics of the tumor, as well as the patient's overall health and wishes. An accurate history and review of pertinent laboratory and prior imaging studies is essential for planning resection of pancreatic neuroendocrine tumors (PNETs).

Genetic background — The patient's history and genetic testing help determine whether the tumor is sporadic or associated with a genetic syndrome. This is important because the association of a genetic syndrome, MEN1 for instance, affects the number and extent of primary PNETs, which greatly impacts surgical decision making [7,8]. The management of PNETs associated with genetic syndromes is discussed separately. (See "Multiple endocrine neoplasia type 1: Treatment" and "Clinical features, diagnosis, and management of von Hippel-Lindau disease" and "Tuberous sclerosis complex: Management and prognosis" and "Neurofibromatosis type 1 (NF1): Management and prognosis".)

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Literature review current through: Nov 2017. | This topic last updated: Mar 16, 2017.
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