angiomyolipoma
angiomyolipoma
[an″je-o-mi″o-lĭ-po´mah]an·gi·o·my·o·li·po·ma
(an'jē-ō-mī'ō-li-pō'mă),angiomyolipoma
(ăn′jē-ō-mī′ō-lĭ-pō′mə)angiomyolipoma
A benign, well-circumscribed, nonencapsulated tumour-like lesion or hamartoma (perivascular epithelioid cell tumour—PEComa), which is the most common mesenchymal lesion of the kidney. It is composed of blood vessels, smooth muscle and mature fat, and is typically seen in the kidney or paranephric tissue. Angiomyolipoma also occurs in the fallopian tube, liver, nasal cavity, penis, skin, spermatic cord and vagina, and can mimic renal cell carcinoma. Up to 50% of patients have tuberous sclerosis (TS); 75% of TS patients have angiomyolipomas.Clinical findings
May cause catastrophic bleeds.
Molecular pathology
TSC1 and TSC2 mutations.