Well-differentiated pancreatic neuroendocrine tumors: From genetics to therapy

Roeland F. De Wilde, Barish H. Edil, Ralph H. Hruban, Anirban Maitra

Research output: Contribution to journalReview articlepeer-review

68 Scopus citations


Well-differentiated pancreatic neuroendocrine tumors (PanNETs) comprise ∼1-3% of pancreatic neoplasms. Although long considered as reasonably benign lesions, PanNETs have considerable malignant potential, with a 5-year survival of ∼65% and a 10-year survival of 45% for resected lesions. As PanNETs have a low incidence, they have been understudied, with few advances made until the completion of their exomic sequencing in the past year. In this Review, we summarize some of the latest insights into the genetics of PanNETs, and their probable implications in the context of prognosis and therapy. In particular, we discuss two genes (DAXX and ATRX) that have collectively been identified as mutated in >40% of PanNETs, and the biological and prognostic implications of these novel mutations. The identification of recurrent somatic mutations within the mTOR signaling pathway and the therapeutic implications for personalized therapy in patients with PanNETs are also discussed. Finally, this Review outlines state-of-the-art advances in the biology of PanNETs that are of emerging translational importance.

Original languageEnglish (US)
Pages (from-to)199-208
Number of pages10
JournalNature Reviews Gastroenterology and Hepatology
Issue number4
StatePublished - Apr 2012

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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