Pancreatic neuroendocrine tumor

Definition

Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. They may occur sporadically or in association with a genetic syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Landau (VHL) syndrome, neurofibromatosis type 1, or tuberous sclerosis. The most frequent genetic alterations in PNET occur in MEN1 (Multiple Endocrine Neoplasia-1 Gene), DAXX/ATRX (Death-Domain Associated Protein/Mental Retardation Syndrome X-Linked Genes) and the mTOR pathway (Mammalian Target of Rapamycin). A germline mutation in the MEN1 tumor suppressor gene causes MEN1, the above-mentioned autosomal dominant hereditary syndrome. Mutations of DAXX and ATRX are common and related to altered telomeres but not to prognosis.

References:

[1]. A Viúdez, et al. Pancreatic neuroendocrine tumors: Challenges in an underestimated disease. Crit Rev Oncol Hematol. 2016 May;101:193-206. [Content Brief]

[2]. Daniel M Halperin, et al. A tale of two tumors: treating pancreatic and extrapancreatic neuroendocrine tumors. Annu Rev Med. 2015;66:1-16. [Content Brief]

[3]. Gabriele Capurso, et al. Molecular pathogenesis and targeted therapy of sporadic pancreatic neuroendocrine tumors. J Hepatobiliary Pancreat Sci. 2015 Aug;22(8):594-601. [Content Brief]

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生物医学辞典は、生物学の学術用語と専門データの包括的で専門的なデータベースです。すべての説明は、権威ある書籍や高いＩＦの文献に基づいており、知りたい生物医学用語の正確な説明を得ることができます。

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