Paraganglioma & PheochromocytomaRSS

Last Updated: November 15, 2013

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What are paraganglioma and pheochromocytoma?

Paraganglioma is a rare cancer that originates in the nerve cells of the adrenal glands, organs on top of each kidney that produce important hormones. Paraganglioma that develops in the center of the adrenal gland is called pheochromocytoma. Paraganglioma that forms outside of the adrenal gland, often along blood vessels and nerves in the head and neck, is called extra-adrenal paraganglioma, or simply paraganglioma.

Each year, between 2 and 8 people per million worldwide are diagnosed with paraganglioma and pheochromocytoma.1 Ten percent of all cases occur in children.2 In both adults and children, pheochromocytoma is more common than paraganglioma.2 No known environmental, dietary, or lifestyle risk factors have been associated with these cancers. However, paraganglioma and pheochromocytoma can be hereditary diseases:3 one study reported that about 41 percent of patients diagnosed with one of these diseases in the U.S. carry inherited genetic mutations that increase the risk of malignancy.1 The Cancer Genome Atlas is studying both hereditary and non-hereditary (sometimes called sporadic) paraganglioma and pheochromocytoma. When the cancer spreads to other parts of the body, only 44 percent of patients survive five years after their diagnosis.2

View additional information on paraganglioma and pheochromocytoma.

Paraganglioma and pheochromocytoma are part of an effort to characterize rare tumor types. Read more about the Rare Tumor Projects.

What types of discoveries about paraganglioma and pheochromocytoma do The Cancer Genome Atlas (TCGA) researchers hope to make?

  • Investigate differences in genomic patterns between paraganglioma and pheochromocytoma tumors
  • Investigate the differences between hereditary and sporadic tumors
  • Characterize the genomic changes that occur in the progression from less to more advanced stage paraganglioma and pheochromocytoma
  • Identify biological molecules that are indicators of malignancy, known as biomarkers, to better detect and diagnose paraganglioma and pheochromocytoma

Where can I find more information about the TCGA Research Network's studies, or studies using TCGA data?

View a list of TCGA scientific publications.

Where can I find clinical trials to treat paraganglioma and pheochromocytoma that are supported by the National Cancer Institute (NCI)?

View a list of NCI-supported  paraganglioma and pheochromocytoma clinical trials that are now accepting patients.

Selected References 

1 Fishbein L, Merrill S, Fraker DL, Cohen DL, and Nathanson KL. (2013) Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 20(5):1444-1450.

2 Hanna NN, and Kenady, DE. (2001) Pheochromocytoma. From: Holzheimer RG, and Mannick JA, eds. Surgical treatment: evidence-based and problem-oriented. Munich: Zuckschwerdt.

3 Genetics Home Reference. (2013) Hereditary paraganglioma-pheochromocytoma. National Library of Medicine.