Overview

NCI Definition: A benign or malignant neuroendocrine neoplasm of the sympathetic nervous system that secretes catecholamines. It arises from the chromaffin cells of the adrenal medulla. Clinical presentation includes headaches, palpitations, chest and abdominal pain, hypertension, fever, and tremor. Microscopically, a characteristic nesting (zellballen) growth pattern is usually seen. Other growth patterns including trabecular pattern may also be present. [1]

Adrenal gland pheochromocytomas most frequently harbor alterations in VHL, ATRX, KMT2D, BRCA2, and NF1 [2].

Most Commonly Altered Genes in Adrenal Gland Pheochromocytoma

VHL Mutation, ATRX Mutation, KMT2D Mutation, BRCA2 Mutation, and NF1 Nonsense are the most common alterations in adrenal gland pheochromocytoma [2].

Top Alterations in Adrenal Gland Pheochromocytoma

Significant Genes in Adrenal Gland Pheochromocytoma

RET +

SDHB +

SDHC +

VHL +

Disease Details

Synonyms
Intraadrenal Paraganglioma, Pheochromocytoma, Adrenal Medullary Paraganglioma, PHEOCHROMOCYTOMA, UNDETERMINED, Adrenal Pheochromocytoma, PCC, Adrenal Gland Chromaffin Paraganglioma, Chromaffin Paraganglioma of the Adrenal Gland, Phaeochromocytoma, Adrenal Gland Chromaffinoma, Pheochromocytoma (adrenal), Adrenal Gland Paraganglioma, Adrenal Medullary Pheochromocytoma
Parent(s)
Sympathetic Paraganglioma
OncoTree Name
Pheochromocytoma
OncoTree Code
PHC

References

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.