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Therapy of metastatic pancreatic neuroendocrine tumors (pNETs): recent insights and advances

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Abstract

Neuroendocrine tumors (NETs) [carcinoids, pancreatic neuroendocrine tumors (pNETs)] are becoming an increasing clinical problem because not only are they increasing in frequency, but they can frequently present with advanced disease that requires diagnostic and treatment approaches different from those used in the neoplasms that most physicians are used to seeing and treating. In the past few years there have been numerous advances in all aspects of NETs including: an understanding of their unique pathogenesis; specific classification systems developed which have prognostic value; novel methods of tumor localization developed; and novel treatment approaches described. In patients with advanced metastatic disease these include the use of newer chemotherapeutic approaches, an increased understanding of the role of surgery and cytoreductive methods, the development of methods for targeted delivery of cytotoxic agents, and the development of targeted medical therapies (everolimus, sunitinib) based on an increased understanding of the disease biology. Although pNETs and gastrointestinal NETs share many features, recent studies show they differ in pathogenesis and in many aspects of diagnosis and treatment, including their responsiveness to different therapies. Because of limited space, this review will be limited to the advances made in the management and treatment of patients with advanced metastatic pNETs over the past 5 years.

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Abbreviations

ACTHomas:

ACTH-secreting pancreatic neuroendocrine tumors

CNS:

Central nervous system

ENETS:

European Neuroendocrine Tumor Society

ESMO:

European Society for Medical Oncology

GI:

Gastrointestinal

GI-NETs:

Gastrointestinal neuroendocrine tumors (carcinoids of gastrointestinal tract)

TAE/TACE:

Transarterial embolization/chemoembolization

IGF:

Insulin-like growth factor

UICC/AJCC/WHO:

International Union for Cancer Control/American Joint Cancer Committee/World Health Organization

MRI:

Magnetic resonance imaging

NANETS:

North American Neuroendocrine Tumor Society

NCCN:

National Comprehensive Cancer Network

NETs:

Neuroendocrine tumors (carcinoids, pancreatic neuroendocrine tumors)

NF-pNETs:

Nonfunctional pancreatic neuroendocrine tumors

pNETs:

Pancreatic neuroendocrine tumors

PDGFRs:

Platelet-derived growth factor receptors

PRRT:

Peptide receptor radionuclide therapy

RFA:

Radiofrequency ablation

SIRT:

Selective internal radiation

SRS:

Somatostatin receptor scintigraphy

VEGFR:

Vascular endothelial growth factor receptor

VIPomas:

Vasoactive intestinal peptide-secreting pancreatic neuroendocrine tumors

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This work was partially supported by intramural funds of NIDDK, NIH.

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Ito, T., Igarashi, H. & Jensen, R.T. Therapy of metastatic pancreatic neuroendocrine tumors (pNETs): recent insights and advances. J Gastroenterol 47, 941–960 (2012). https://doi.org/10.1007/s00535-012-0642-8

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