| Angiomyolipoma | |
|---|---|
 | |
| Angiomyolipoma in both kidneys (arrows) as seen on CT scan. The tumors are hypodense (dark) due to fat content. | |
| Specialty | Nephrology |
| Symptoms | None, blood in the urine, pain[1] |
| Complications | Hemorrhagic shock, kidney failure[2][3] |
| Usual onset | 20 to 50 years[3] |
| Causes | Tuberous sclerosis, lymphangioleiomyomatosis, spontaneous[1][3] |
| Diagnostic method | CT scan or MRI[3] |
| Differential diagnosis | Renal cell cancer[3] |
| Treatment | Surveillance, surgical removal, embolisation,ablation[3] |
| Frequency | Uncommon[3] |
Angiomyolipoma (AML) is a benign tumor of fat, blood vessels, and muscle tissue, that is usually found in the kidney.[1][2] They rarely cause symptoms, but may occasionally result in blood in the urine or grow to a size that pain occurs.[1][3] Complications can include hemorrhagic shock and kidney failure.[2][3] Occasionally other organs like the liver may be affected.[2]
They occur in up to 90% of cases of tuberous sclerosis (TSC) and 50% of cases of lymphangioleiomyomatosis (LAM).[1][3] In tuberous sclerosis often multiple lesions are present.[3] They may also occur spontaneously.[3] They can usually be diagnosed by CT scan or MRI.[3]
Small angiomyolipoma that do not result in symptoms can often we simple watched.[3] Larger or symptomatic cases may be treated by surgical removal, embolisation, or ablation.[3] In cases due to TSC or LAM, mTOR inhibitors may be an option.[3] Occasionally dialysis or kidney transplant is required.[2] They are uncommon.[3] Females are more commonly affected than males.[3] Onset is generally between 20 and 50 years of age.[3] It was first described in 1900.[3]
References
edit- ^ a b c d e "NCI Search Results - National Cancer Institute". www.cancer.gov. 2 February 2011. Archived from the original on 27 August 2021. Retrieved 26 January 2021.
- ^ a b c d e Northrup, H; Krueger, DA (2013). "International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference". Pediatr Neurol. 49 (4): 243–254. doi:10.1016/j.pediatrneurol.2013.08.001. PMC 4080684. PMID 24053982.
- ^ a b c d e f g h i j k l m n o p q r s t Flum, AS; Hamoui, N; Said, MA; Yang, XJ; Casalino, DD; McGuire, BB; Perry, KT; Nadler, RB (April 2016). "Update on the Diagnosis and Management of Renal Angiomyolipoma". The Journal of urology. 195 (4 Pt 1): 834–46. doi:10.1016/j.juro.2015.07.126. PMID 26612197.