Well-differentiated pancreatic neuroendocrine tumors: from genetics to therapy

Roeland F de Wilde, Barish H Edil, Ralph H Hruban, Anirban Maitra*

*Corresponding author for this work

Research output: Contribution to journalReview articleAcademicpeer-review

90 Citations (Scopus)

Abstract

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
Pages (from-to)199-208
Number of pages10
JournalNature Reviews Gastroenterology and Hepatology
Volume9
Issue number4
DOIs
Publication statusPublished - 7 Feb 2012
Externally publishedYes

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