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Genetics of Pancreatic Neuroendocrine Tumors
Chirayu Mohindroo1, Florencia McAllister2, Ana De Jesus-Acosta3
1Department of Clinical Cancer Prevention, The University of Texas MD Anderson Cancer Center, 1515 Holcombe, Unit 1360, Houston, TX 77030, USA; Department of Internal Medicine, Sinai Hospital of Baltimore, 2435 W. Belvedere Ave, Ste 56, Baltimore, MD 21215, USA.
Abstract:
Pancreatic neuroendocrine tumors (pNETs) represent a relatively rare disease; however, the incidence has been increasing during the last 2 decades. Next generation sequencing has greatly increased our understanding of driver mutations in pNETs. Sporadic pNETs have consistently presented with mutations in MEN1, DAXX/ATRX, and genes related to the mammalian target of rapamycin pathway. Inherited pNETs have traditionally been associated with multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, neurofibromatosis type 1, and tuberous sclerosis complex. The current review expands on the existing knowledge and the relevant updates on the genetics of pNETs.
Insights
Pancreatic neuroendocrine tumors (pNETs) are increasingly diagnosed. Genetic analysis reveals key mutations like MEN1 and DAXX/ATRX in sporadic pNETs, and links inherited forms to specific syndromes.
Area of Science:
- Oncology
- Genetics
- Endocrinology
Background:
- Pancreatic neuroendocrine tumors (pNETs) incidence is rising.
- Next-generation sequencing advances pNETs genetic understanding.
Purpose of the Study:
- To review and update current knowledge on pNETs genetics.
- To highlight driver mutations in sporadic and inherited pNETs.
Main Methods:
- Literature review of genetic studies in pNETs.
- Analysis of next-generation sequencing data.
Main Results:
- Sporadic pNETs commonly show MEN1, DAXX/ATRX, and mTOR pathway gene mutations.
- Inherited pNETs are associated with MEN1, VHL, NF1, and TSC.
Conclusions:
- Genetic landscape of pNETs is complex and evolving.
- Understanding pNETs genetics is crucial for diagnosis and treatment.
