CANCER
GENETIC CANCER RISK
Endocrine
High Risk
Other
Elevated Risk
Renal
Elevated Risk
CANCER TYPE
PROCEDURE
CANCER RISK
RISK FOR GENERAL POPULATION *
Paraganglioma/Pheochromocytoma
To age 60
10% risk for tumors, only some of which will be malignant
<0.1%
Renal
To age 80
Elevated risk
1.4%
Gastrointestinal Stromal Tumors (GIST)
To age 80
Elevated risk
<0.1%
CANCER TYPE
PROCEDURE
AGE TO BEGIN
FREQUENCY
Paraganglioma/Pheochromocytoma
Clinical monitoring, including blood pressure measurement
6 to 8 years
Annually
Biochemical screening of serum and urine
6 to 8 years
Annually
Whole-body MRI with consideration of the head and neck
6 to 8 years
Every 2 years
Renal
Abdominal MRI (preferred) or CT, with and without IV contrast
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.
6 to 8 years
Annually
SDHA
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 Recessive
Prevalence
Unknown