doi: 10.5732/cjc.012.10295

Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment

Cynthia Ro, Wanxing Chai, Victoria E. Yu, Run Yu

Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA.

[Abstract] Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. A 4-pronged aggressive treatment approach consisting of surgery, locoregional therapy, systemic therapy, and complication control has become popular in academic centers around the world. The optimal application of the multiple systemic therapeutic modalities is under development; efficacy, safety, availability, and cost should be considered when treating a specific patient. The clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including the novel Mahvash disease, are summarized.

Chinese Journal of Cancer 2013, Volume: 32, Issue 6, Page: 312-324

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Cynthia Ro, Wanxing Chai, Victoria E. Yu, Run Yu. Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment. Chin J Cancer. 2013, 32(6):312-324. doi:10.5732/cjc.012.10295


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