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Endocrinology

Neuroendocrine Tumors of the Pancreas

^aDepartment for General, Thoracic and Vascular Surgery, University Hospital "Carl Gustav Carus," University of Technology, Dresden, Germany; ^bDepartment of Surgery, Indiana University School of Medicine, Indianapolis, Indiana, USA

Key Words. Pancreas • Neuroendocrine • Tumor • Insulinoma • Gastrinoma

Correspondence: Robert Grützmann, M.D., Department for General, Thoracic and Vascular Surgery, University Hospital "Carl Gustav Carus," University of Technology, Fetscherstraße 74, 01307 Dresden, Germany. Telephone: +49 351 458 6996; Fax: +49 351 458 4395; e-mail: Robert.Gruetzmann{at}uniklinikum-dresden.de

Received November 23, 2008; accepted for publication March 27, 2009; first published online in THE ONCOLOGIST Express on May 1, 2009.

Disclosures

Florian Ehehalt: None; Hans D. Saeger: None; C. Max Schmidt: None; Robert Grützmann: None.

Section editors Herbert Chen and Stan B. Sidhu have disclosed no financial relationships relevant to the content of this article.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias.

This literature review briefly summarizes the epidemiology, pathophysiology, clinical management, and outcomes of patients with pancreatic neuroendocrine tumors (PNETs) and highlights recent advances in PNET research. PNETs are rare neoplasms, compared with carcinomas arising from pancreatic exocrine tissue. They, like other neuroendocrine tumor types, display variable malignant potential, hormone-related syndromes (functionality), localization, and genetic background. Although tumor origin and molecular pathogenesis remain poorly understood, recently established grading and staging systems facilitate patient risk stratification, and thereby directly impact clinical decision making.

Although the optimal clinical management of PNETs involves a multidisciplinary approach, surgery remains the only curative treatment for early-stage disease. Surgery may also have a role in patients with advanced-stage disease, including those with hepatic metastases. Alternative therapeutic approaches applied to PNETs, including chemotherapy, radiofrequency ablation, transarterial chemoembolization, biotherapy, polypeptide radionuclide receptor therapy, antiangiogenic therapy, and selective internal radiotherapy, have failed to demonstrate a long-term survival benefit. Surgery remains the primary therapeutic option for patients with PNETs. Research on PNETs is desperately needed to improve the therapeutic options for patients with this disease.