Abstract
Pancreatic neuroendocrine tumors (PNETs) are rare neoplasms. They are clinically diverse and divided into functioning and nonfunctioning disease, depending on their ability to produce symptoms due to hormone production. Surgical resection is the only curative treatment and remains the cornerstone therapy for this patient group, even in patients with advanced disease. Over the last decade there has been a noticeable trend towards more aggressive surgery as well as more minimally invasive surgery in patients with PNETs. This has resulted in improved long-term survival in patients with locally advanced and metastatic disease treated aggressively, as well as shorter hospital stays and comparable long-term outcomes in patients with limited disease treated minimally invasively. There are still controversies related to issues of surgical treatment of PNETs, such as to what extent enucleation, lymph node sampling, and vascular reconstruction are beneficial for the oncologic outcome. Histopathologic tumor classification is of high clinical importance for treatment planning and prognostic evaluation of patients with PNETs. A constant challenge, which relates to the treatment of PNETs, is the lack of an internationally accepted histopathological classification system. This paper reviews current issues on the surgical treatment of sporadic PNETs with specific focus on surgical approaches and tumor classification.
Highlights
Clinical PresentationPancreatic neuroendocrine tumors (PNETs) are rare and account for about 1-2% of all pancreatic neoplasms [1, 2]
Pancreatic neuroendocrine tumors (PNETs) are rare neoplasms
There are still controversies related to issues of surgical treatment of PNETs, such as to what extent enucleation, lymph node sampling, and vascular reconstruction are beneficial for the oncologic outcome
Summary
Pancreatic neuroendocrine tumors (PNETs) are rare and account for about 1-2% of all pancreatic neoplasms [1, 2]. Autopsy studies have shown that PNETs can be identified in as many as 10% of the population, suggesting that many carry asymptomatic disease [6]. PNETs are clinically diverse and divided into functioning and nonfunctioning disease, dependent on their ability to produce symptoms due to hormone production [7]. The distinction between nonfunctioning and functioning PNETs is based on immunohistochemistry of tumor tissue in addition to clinical symptoms. Common symptoms of nonfunctioning PNETs are abdominal pain, nausea and/or vomiting, fatigue, obstructive. Patients with functioning PNETs, such as insulinoma and gastrinoma, often present with characteristic symptoms dependent on the hormones produced. The clinical relevance of the distinction between functioning and nonfunctioning PNETs has recently been questioned as the treatment of these tumors follow the same general principles [13]
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