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. [1]

Paragangliomas most frequently harbor alterations in ATRX, SDHB, KMT2D, VHL, and BRCA1 [2].

Most Commonly Altered Genes in Paraganglioma

ATRX Mutation, KMT2D Mutation, VHL Mutation, SDHB Mutation, and BRCA1 Mutation are the most common alterations in paraganglioma [2].

Top Alterations in Paraganglioma

Significant Genes in Paraganglioma

FH +






Disease Details

Paraganglion Neoplasm, Paraganglionic Neoplasm, Paraganglionic Tumor, Neoplasm of Paraganglion, Neoplasm of the Paraganglion, PARAGANGLIOMA, UNDETERMINED, Tumor of Paraganglion, Tumor of the Paraganglion, Paraganglion Tumor, Soft tissue paraganglioma
Neuroendocrine Neoplasm
Malignant Paraganglioma, Sympathetic Paraganglioma, and Extra-Adrenal Paraganglioma
OncoTree Name
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

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.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.