Pheochromocytoma and Paraganglioma: Other FAQs

There are common questions that we can answer about all health topics, such as "What is it?" and "How many people are affected." Answers to these questions are found under Condition Information. Each health topic frequently has specific questions that pertain only to that topic. We have answered those in this section.

What is the prognosis for patients with benign pheochromocytoma?

The prognosis for patients with a completely removed tumor is excellent.¹ The 5-year survival rate for patients with benign pheochromocytomas is greater than 95%.²

What is the prognosis for patients with malignant pheochromocytoma?

In patients with malignant pheochromocytomas, the 5-year survival rate is less than 50%.² Life expectancy varies for patients with malignant pheochromocytoma depending on where the tumor has spread. Survival is generally less than 5 years when tumors have spread to the liver and lung and somewhat longer when tumors have spread to the bones. ³

Are there disorders or conditions associated with pheochromocytoma?

Pheochromocytoma can be inherited as part of another syndrome or may coexist with other diseases. Some patients with pheochromocytoma have the inheritable disease von Hippel-Lindau (VHL) syndrome or neurofibromatosis type 1 (NF1).⁴ Both VHL and NF1 are cancer syndromes in which patients have tumors at multiple sites.

Another disease in which pheochromocytoma is often present is multiple endocrine neoplasia type 2 (MEN2), which produces tumors in the endocrine glands, including the parathyroid gland.⁴ Approximately 50% of patients with MEN2 have pheochromocytoma.⁴

Are there any gender or race differences in the proportion of cases?

Pheochromocytomas are equally common in males and females.⁵

Pheochromocytomas are diagnosed in people of all races but are less common in African Americans.5

Could pheochromocytoma affect pregnancy?

Approximately 30% of pheochromocytoma cases are inheritable and the remaining cases occur sporadically.⁴ Children are at risk of developing pheochromocytoma/paraganglioma if they inherit one of the known disease-causing mutated genes from their parents. The penetrance of the disease, or the percentage of individuals with a known genetic mutation who develop the disease, varies with different mutations.

Pregnancy may be complicated by the clinical signs of pheochromocytoma. In pregnant women, high blood pressure, cardiovascular problems, and seizures, which occur in patients with pheochromocytoma,⁵ could affect the health of the unborn child.

Is genetic testing available for pheochromocytoma?

Yes. Genetic testing is available for pheochromocytoma. Genetic testing is especially recommended for some groups of patients who⁴:

• Have a family history of pheochromocytoma
• Have clinical features of syndromes associated with pheochromocytoma
• Have multiple tumors or an extra-adrenal tumor (tumor is at a location other than the adrenal glands) (paraganglioma)
• Are diagnosed before age 50
• Have specific biochemical results

Genetic testing may also be recommended for other patients depending upon family history, the patient's age, and the location of the patient's tumor.⁶

What should we do if our family has tested positively for a genetic mutation associated with pheochromocytoma?

Disease screening should be offered to presymptomatic relatives who have a diagnosed mutation, especially because familial syndromes are also associated with other types of tumors and early diagnosis improves the prognosis of these patients. Patients with known genetic mutations should undergo regular periodic screening with biochemical testing and anatomic imaging (CT or MRI scans) to rule out the presence of disease.

1. Lenders, J. W., Eisenhofer, G., Mannelli, M., & Pacak, K. (2005.) Phaeochromocytoma. Lancet 366: 665-675. [top]
2. Blake, M. A. (2011). Pheochromocytoma. Retrieved April 5, 2012, from http://emedicine.medscape.com/article/124059-overview [top]
3. Waguespack, S. G., Rich, T., Grubbs, E., Ying, A. K., Perrier, N. D., Ayala-Ramirez, M., et al. (2010) A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. Journal of Clinical Endocrinology and Metabolism 95: 2023-2037. [top]
4. Karasek, D., Shah, U., Frysak, Z., Stratakis, C., Pacak, K. (In press). An update on the genetics of pheochromocytoma. Journal of Human Hypertension , doi:10.1038/jhh.2012.20. [top]
5. Eunice Kennedy Shriver National Institute of Child Health and Human Development. The proper diagnosis, treatment, genetics, and research of pheochromocytoma and paraganglioma: Overview. Retrieved March 16, 2012, from https://science.nichd.nih.gov/confluence/display/pheo/Overview [top]
6. National Cancer Institute. Pheochromocytoma and paraganglioma treatment (PDQ). Retrieved March 7, 2012, from http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0033247/ [top]