Endocrine

Other

Elevated Risk

Renal

Elevated Risk

CANCER RISK

Paraganglioma/Pheochromocytoma

To age 60

25% risk for tumors, only some of which will be malignant

<0.1%

Renal

Elevated risk

1.4%

Gastrointestinal Stromal Tumors (GIST)

Elevated risk

<0.1%

Paraganglioma/Pheochromocytoma

Clinical monitoring, including blood pressure measurement3, 4

6 to 8 years

Annually

Biochemical screening of serum and urine3, 4

6 to 8 years

Annually

Whole-body MRI with consideration of the head and neck3, 4

6 to 8 years

Every 2 years

Renal

Abdominal MRI (preferred) or CT, with and without IV contrast1

12 years

Every 4 to 6 years

Gastrointestinal Stromal Tumors (GIST)

Complete blood count and attention to symptoms such as gastric bleeding, obstruction, abdominal pain, nausea, etc.4

6 to 8 years

Annually

Associated Syndrome

Hereditary Pheochromocytoma-Paraganglioma syndrome (Hereditary PPGL syndrome)

Core Cancer Risk(s)

Paragangliomas and Pheochromocytomas (that often secrete hormones that cause high blood pressure, rapid and/or abnormal heartbeat, headaches, sweating, nausea, fatigue, and anxiety). Elevated Risk for renal cancer and gastrointestinal stromal tumors

Inheritance 

Autosomal Dominant 

Prevalence

Unknown