TSC1

Associated Syndrome

Tuberous Sclerosis Complex

Core Cancer Risk(s)

Renal Cancer and Malignant Angiomyolipoma

Inheritance 

Autosomal Dominant 

Prevalence

Unknown

TSC2

Associated Syndrome

Tuberous Sclerosis Complex

Core Cancer Risk(s)

Renal Cancer and Malignant Angiomyolipoma

Inheritance 

Autosomal Dominant 

Prevalence

Unknown

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CANCER

GENETIC CANCER RISK

Other

High Risk

Renal

Elevated Risk

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CANCER TYPE

PROCEDURE

CANCER RISK

RISK FOR GENERAL POPULATION *

Renal

To age 80

2%-5%

1.4%

Malignant Angiomyolipoma

To age 80

Rare, but elevated risk

<0.1%

Other - Non-malignant features of TSC

All ages

TSC is associated with many life-threatening non-malignant clinical features, some of which may require medical intervention as early as infancy (see Overview).

NA

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CANCER TYPE

PROCEDURE

AGE TO BEGIN

FREQUENCY

Renal

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

12 years

Every 3 to 5 years

Malignant Angiomyolipoma

Abdominal MRI

At time of diagnosis of TSC

Every 1 to 3 years

Consider everolimus therapy for asymptomatic, growing angiomyolipoma measuring >3 cm in diameter

Individualized

NA

Other - Non-malignant features of TSC

Multiple screenings recommended, which may include comprehensive physical exam, brain MRI, echocardiogram, chest CT, and evaluations for neuropsychiatric disorders.

At time of diagnosis of TSC

Varies