ANGIOMYOLIPOMA
Angiomyolipoma are the common noncancerous (benign) tumours of the kidney.
- They are composed of three types of cells: the vascular cells, fat cells, and immature smooth muscle cells.
- Although they are benign, angiomyolipoma may grow to impair kidney function or may cause the blood vessels to dilate and burst, causing bleeding.
- They are mostly found in the kidneys, but they can be occasionally found in the liver, and rarely in the ovary, colon, fallopian tube, spermatic plate.
- Angiomyolipoma affects both sexes and affects young adults between ages 30 to 40.
- Women tend to have more numerous and larger angiomyolipoma than men
CAUSES
Angiomyolipoma is caused by a mutation in either the TSC1 or TSC2 cells, which controls cell growth and proliferation.
They are also associated with tuberous sclerosis, a genetically inherited disease. Tuberous sclerosis is a rare genetic condition that causes benign tumors to grow in the brain and other organs. Angiomyolipoma is evident in 80% tuberous sclerosis patient by age 10 years.\
They can also be found in women with lymphangioleiomyomatosis.
SYMPTOMS
Angiomyolipoma that is less than 4cm in diameter produces no symptom (asymptomatic). Symptoms may be present if the angiomyolipoma exceeds 4cm in diameter, and may include:
- Acute or chronic abdominal or flank pain
- Nausea
- Vomiting
- Hypertension
- Chronic kidney disease
- Shock, seen in about 20% of the patient that show symptoms
DIAGNOSIS AND TREATMENT
The diagnosis of the condition is done incidentally while the patient is ongoing imaging tests for other reasons, this is because many are asymptomatic.
The condition can also be diagnosed directly by using imaging studies including CT scan, ultrasound, and/or MRI scans.
TREATMENT
The condition can be treated using the drug everolimus (Afinitor). This drug is FDA approved
Asymptomatic angiomyolipoma that grows to 3cm or more in diameter should be routinely seen with follow-up measurements of the tumor size, as they may rapidly grow up to 4cm.
Another treatment option is embolization, where the blood vessels in angiomyolipoma are sealed off.
Surgery is only required for a severe condition that involves life-threatening bleeding,