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
CANCER TYPE
AGE RANGE
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
CANCER TYPE
PROCEDURE
AGE TO BEGIN
FREQUENCY
Renal
Abdominal imaging with MRI (preferred) or CT, with and without contrast1
12 years, or individualized to a younger age based on the earliest renal cancer diagnosis in the family
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