Multiple angiomyolipoma from a contrast-enhanced CT of the abdomen of a patient with TSC.
Angiomyolipoma is a benign renal lesion. It is composed of fat, vascular, and smooth muscle elements. It occurs in more than 50% of individuals with tuberous sclerosis. It is twice as common in females as in males. The fat density of the tumour on CT is pathognomonic. Image File history File links Please see the file description page for further information. ... Image File history File links Please see the file description page for further information. ... Tuberous sclerosis is a rare genetic disorder characterized by a triad of signs: seizures, mental retardation, and small benign facial skin tumors (angiofibromas). ... It has been suggested that Renal anomalies and Renal plasma threshold be merged into this article or section. ... For other uses, see Fat (disambiguation). ... Vascular is an adjective for the word vessel and refers to tube-like structures. ... Cultured Smooth muscle of the aorta. ... Tuberous sclerosis is a rare genetic disorder characterized by a triad of signs: seizures, mental retardation, and small benign facial skin tumors (angiofibromas). ... Look up CT, Ct, ct in Wiktionary, the free dictionary. ... Pathognomonic is a word, often used in medicine, which means characteristic or diagnostic for a particular disease. ...
Conversely, the prevalence of angiomyolipoma in the general population, tuberous sclerosis and lymphangiomyomatosis is 0.3%, 80% and 15%, respectively.
Angiomyolipoma in the general population is diagnosed at a mean age of 45, is bilateral in 10% of cases, and has a female : male ratio of 4:1.
Angiomyolipoma in tuberous sclerosis is diagnosed at a mean age of 17, is bilateral in 80% of cases, and has a female : male ratio of 1:1.
Angiomyolipoma a complex neoplasm typically composed of thick-walled blood vessels, smooth muscle, and fat, the relative proportions of which vary widely When fat predominates, angiomyolipoma is yellow and has a greasy appearance.
Sporadic angiomyolipomas are single and tend to be larger than 4 cm in diameter since the fat density on CT scan facilitates the radiologic diagnosis and it is recommended that asymptomatic ones be removed only when they exceed 4 cm (rupture and hemorrhage are uncommon in smaller ones).
Epithelioid angiomyolipoma uniformly fails to react with antibodies to cytokeratins and epithelial membrane antigen, and reacts with antibodies to smooth muscle actin, and to a lesser extent, desmin.
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