medical_managment-key.png

CANCER

GENETIC CANCER RISK

Endocrine 

High Risk

Other

High Risk

Renal

High Risk

medical_managment-key.png

CANCER TYPE

PROCEDURE

AGE TO BEGIN

RISK FOR GENERAL POPULATION *

Renal

To age 60

17%-70%

0.5%

Pancreatic Neuroendocrine Tumors (PNET)

To age 75

5%-17% for tumors, only some of which will be malignant

<0.1%

Paraganglioma/Pheochromocytoma

To age 75

10%-25% for tumors, only some of which will be malignant

<0.1%

Other - Non-malignant features of VHL

All ages

VHL is associated with a high risk for a wide range of non-malignant clinical features, some of which require medical intervention in early childhood.

NA

medical_managment-key.png

CANCER TYPE

PROCEDURE

AGE TO BEGIN

FREQUENCY

Renal

Abdominal imaging with MRI (preferred) or CT, with and without contrast

10 to 15 years

Every 1 to 2 years

Pancreatic Neuroendocrine Tumors (PNET)

Abdominal imaging with MRI (preferred) or CT, with and without contrast

10 to 15 years

Every 1 to 2 years

Paraganglioma/Pheochromocytoma

Blood pressure monitoring

2 years

Annually or at any medical visit

Biochemical monitoring of blood and urine

2 to 5 years

Annually or prior to any surgical procedure

Abdominal imaging with MRI (preferred) or CT, with and without contrast

15 years

Every 2 years

Other - Non-malignant features of VHL

Multiple screenings are recommended, including MRI of the brain, spine and abdomen, vision and hearing exams, neurological assessment, blood and urine tests

Some screenings are recommended from birth

Varies, but most are annually