Associated Genetic Biomarkers
NCI Definition: A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases. 
Paragangliomas most frequently harbor alterations in ATRX, SDHB, KMT2D, VHL, and BRCA1 .
ATRX Mutation, KMT2D Mutation, VHL Mutation, SDHB Mutation, and BRCA1 Mutation are the most common alterations in paraganglioma .
There are 20 clinical trials for paraganglioma, of which 16 are open and 4 are completed or closed. Of the trials that contain paraganglioma as an inclusion criterion, 5 are phase 1/phase 2 (4 open) and 15 are phase 2 (12 open).
SSTR2, SSTR1, and SSTR3 are the most frequent gene inclusion criteria for paraganglioma clinical trials .
Lutetium lu 177 dotatate, cabozantinib, and iobenguane i-131 are the most common interventions in paraganglioma clinical trials.
Significant Genes in Paraganglioma
SDHC is an inclusion eligibility criterion in 2 clinical trials for paraganglioma, of which 0 are open and 2 are closed. Of the trials that contain SDHC status and paraganglioma as inclusion criteria, 2 are phase 2 (0 open) .
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.