Take our online questionnaire to find out if genetic counseling is appropriate for you.

< Back to disease list

Hereditary paraganglioma-pheochromocytoma syndromes

Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are caused by inherited mutations in the SDHD, SDHC, and SDHB genes. These gene mutations lead to the development of multiple neuroendocrine tumors along the brain and spinal cord (paraganglioma) and paraganglioma tumors in the adrenal gland (pheochromocytoma), both of which can be cancerous. Although not everyone with PGL/PCC will get cancer, having these conditions significantly increases the risk to develop certain cancers.

Features of the condition

• Paraganglioma
• Pheochromocytoma (adrenal gland tumor)
• Stomach cancer (specifically gastrointestinal stromal tumors [GIST])
• Kidney cancer
• Thyroid cancer

Risk Factors

• Family history
• Individuals with paraganglioma or pheochromocytoma tumors that are
• Multiple (more than one separate tumor), multifocal, or recurrent tumors
• Early onset (younger than age 40)
• More than one family member with paraganglioma or pheochromocytoma

Genetic Tests

The following genetic tests may be appropriate based on your personal and/or family history:

• SDHD
• SDHB
• SDHC

Schedule an Appointment

To speak with a board-certified genetic counselor about your risks for this disease:

• Schedule genetic counseling appointment online
• Or call 1-800-975-4819
• Send email

Learn More

Genereviews