Angiomyolipoma
| Angiomyolipoma | |
|---|---|
 |
|
| Angiomyolipoma in both kidneys (arrows) in computer tomography. The tumours are hypodense (dark) due to fat content | |
| Classification and external resources | |
| Specialty | oncology |
| ICD-10 | D30.0 |
| ICD-9-CM | 223.0 |
| ICD-O | M8860/0 |
| DiseasesDB | 29496 |
| MeSH | D018207 |
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.
Angiomyolipomas are strongly associated with the genetic disease tuberous sclerosis, in which most individuals will have several angiomyolipomas affecting both kidneys. They are also commonly found in women with the rare lung disease lymphangioleiomyomatosis. Angiomyolipomas are less commonly found in the liver and rarely in other organs. Whether associated with these diseases or sporadic, angiomyolipomas are caused by mutations in either the TSC1 or TSC2 genes, which govern cell growth and proliferation. They are composed of blood vessels, smooth muscle cells and fat cells.
Large angiomyolipoma can be treated with embolisation. Drug therapy for angiomyolipoma is at the research stage. The Tuberous Sclerosis Alliance has published guidelines on diagnosis, surveillance and management.
If the dilated blood vessels in an angiomyolipoma rupture, the resulting retroperitoneal haemorrhage causes sudden pain, accompanied with nausea and vomiting. When the patient presents in the emergency department, up to 20% will be in shock.
Angiomyolipomas are tumours consisting of perivascular epithelioid cells (cells which are found surrounding blood vessels and which resemble epithelial cells). A tumour of this kind is known as a PEComa, from the initials of perivascular epithelioid cell. Older literature may classify them as hamartomas (benign tumours consisting of cells in their correct location but forming a disorganised mass) or choristoma (benign tumours consisting of normal cells in the wrong location). PEComas are themselves a kind of mesenchymal tumour which involves cells that form the connective tissue, cardiovascular and lymphatic systems.
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