Angiomyolipoma

Presentation :

• Renal angiomyolipoma are typically found incidentally on radiological imaging. When symptomatic often have flank pain, gross hematuria, and retroperitoneal hemorrhage.
• Most often occurs sporadically (>50% of pts w/ renal and 90-95% of pts w/ hepatic tumors) but highly associated with Tuberous Sclerosis (50-80% of pts develop these tumors)
  • 4:1 female to male predominance in sporadic cases; 1:1 in Tuberous Sclerosis
• Typical form of this tumor is a benign lesion with essentially no capacity for metastasis. There is however an epithelioid angiomyolipoma which behaves as a malignant neoplasm and can metastasize (~33% of cases)

Pathophysiology :

• Benign clonal mesenchymal neoplasm that most commonly arises in the kidney, or less commonly the liver and very rarely at other anatomical sites
• Hereditary cases
• Sporadic cases are thought to result from spontaneous somatic mutations

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