How does tuberous sclerosis affect the kidneys?
How does tuberous sclerosis affect the kidneys?
People who have this condition tend to develop many cysts that grow and ultimately crowd renal tissue and compromise kidney function. Because renal cysts can occur in people with polycystic kidney disease who do not have TSC, they are considered a minor feature in the diagnostic criteria for TSC.
What renal abnormalities are found in patients with tuberous sclerosis?
The majority of individuals (greater than 80%) with tuberous sclerosis complex (TSC) will develop some form of renal (kidney) disease during their lifetime. There are three particular renal disorders in TSC: renal cysts, renal angiomyolipoma and renal cell carcinoma.
Which kidney tumor is associated with tuberous sclerosis?
Renal cell carcinoma (RCC) occurs in 2-4% of patients with tuberous sclerosis complex (TSC). Previous reports have noted a variety of histologic appearances in these cancers, but the full spectrum of morphologic and molecular features has not been fully elucidated.
What is tuberous sclerosis syndrome?
Tuberous sclerosis, also known as tuberous sclerosis complex, is a rare genetic condition that causes mainly non-cancerous (benign) tumours to develop in different parts of the body. The tumours most often affect the brain, skin, kidneys, heart, eyes and lungs.
Does angiomyolipoma affect kidney function?
Angiomyolipomas are the most common benign tumour of the kidney. Although regarded as benign, angiomyolipomas may grow such that kidney function is impaired or the blood vessels may dilate and burst, leading to bleeding.
What is renal angiomyolipoma?
Angiomyolipoma (AML) is a benign renal neoplasm composed of fat, vascular, and smooth muscle. Two types are described: isolated angiomyolipoma and angiomyolipoma that is associated with tuberous sclerosis (see the images below). AML that is associated with tuberous sclerosis accounts for 20% of angiomyolipomas.
Is tuberous sclerosis rare?
Tuberous sclerosis is a rare genetic disorder that affects 1 in 6,000 newborns in the United States. Approximately 40,000 to 80,000 people in the United States have tuberous sclerosis.