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• W3007888461 endingPage "T52" @default.
• W3007888461 startingPage "T41" @default.
• W3007888461 abstract "Pheochromocytomas and paragangliomas (PPGLs) are adrenal or extra-adrenal autonomous nervous system-derived tumors. Most PPGLs are benign, but approximately 15% progress with metastases (mPPGLs). mPPGLs are more likely to occur in patients with large pheochromocytomas, sympathetic paragangliomas, and norepinephrine-secreting tumors. Older subjects, those with larger tumors and synchronous metastases, advance more rapidly. Germline mutations of SDHB , FH , and possibly SLC25A11 , or somatic MAML3 disruptions relate to a higher risk for metastatic disease. However, it is unclear whether these mutations predict outcome. Once diagnosed, there are no well-established predictors of outcome in mPPGLs, and aggressive tumors have few therapeutic options and limited response. High-specific activity (HSA) metaiodine-benzyl-guanidine (MIBG) is the first FDA approved treatment and shows clinical effectiveness for MIBG-avid mPPGLs. Ongoing and future investigations should involve validation of emerging candidate outcome biomarkers, including somatic ATRX , TERT , and microRNA disruptions and identification of novel prognostic indicators. Long-term effect of HSA-MIBG and the role of other radiopharmaceuticals should be investigated. Novel trials targeting molecular events prevalent in SDHB / FH mutant tumors, such as activated hypoxia inducible factor 2 (HIF2), angiogenesis, or other mitochondrial defects that might confer unique vulnerability to these tumors should be developed and initiated. As therapeutic options are anticipated to expand, multi-institutional collaborations and well-defined clinical and molecular endpoints will be critical to achieve higher success rates in improving care for patients with mPPGLs." @default.
• W3007888461 created "2020-03-06" @default.
• W3007888461 creator A5002174272 @default.
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• W3007888461 creator A5088837419 @default.
• W3007888461 date "2020-08-01" @default.
• W3007888461 modified "2023-10-16" @default.
• W3007888461 title "HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES: Metastatic pheochromocytomas and paragangliomas: proceedings of the MEN2019 workshop" @default.
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• W3007888461 doi "https://doi.org/10.1530/erc-19-0435" @default.
• W3007888461 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7334096" @default.
• W3007888461 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/32069214" @default.
• W3007888461 hasPublicationYear "2020" @default.
• W3007888461 type Work @default.

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