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CANCER

GENETIC CANCER RISK

Endocrine

High Risk

Other

High Risk

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

RISK FOR GENERAL POPULATION *

Medullary Thyroid

Lifetime risk

Up to 100%

<0.1%

Pheochromocytoma

To age 80

Up to 50%

<0.1%

Other MEN2A, FMTC and MEN2B-associated tumors and cancers

To age 80

Depends on gene mutation

NA

RET

Associated Syndrome

Multiple Endocrine Neoplasia Type 2A (MEN2A), Familial Medullary Thyroid Cancer (FMTC), and Multiple Endocrine Neoplasia Type 2B (MEN2B)

Core Cancer Risk(s)

Medullary Thyroid Cancer

Inheritance 

Autosomal Dominant 

Prevalence

Unknown

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

FREQUENCY

Medullary Thyroid

Thyroidectomy

ndividualized, based on risk associated with specific RET mutations (infancy, early childhood, or after screening detects signs of thyroid cancer)

NA

Screening with biochemical testing, physical exam and thyroid ultrasound for patients with lower risk mutations

Age 6 months to 5 years

Every 6 months to annually

Pheochromocytoma

Biochemical screening of blood and urine

Individualized, based on risk associated with specific RET mutations (age 8 to 16)

Annually

Abdominal/pelvic MRI (preferred) or CT

If biochemical screening is abnormal

Individualized

Other MEN2A, FMTC and MEN2B-associated tumors and cancers

Biochemical screening for hypercalcemia for individuals with RET mutations associated with hyperparathyroidism

Age 11 to 16

Annually

Additional clinical monitoring for clinical features of MEN2A and MEN2B based on mutation identified

Individualized

Individualized