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Harmatoma:


DefinitionAngiomyolipoma is a benign renal neoplasm previously considered to be a hamartoma or choristoma, but now known to be neoplastic. It is composed of variable amounts of fat, vascular, and smooth muscle elements.

Clinical Features
*Usually adult
*May:
1.be found incidentally
2.result in retroperitoneal hemorrhage, which can be massive and even fatal
3.be associated with tuberous sclerosis:
≈33% of patients, the incidence being higher if the tumors are multiple or bilateral)
≈80% of patients with the complete or severe form of tuberous sclerosis have renal angiomyolipomas
4.be associated with the genetic disease known as TSC2/PKD1 contiguous gene syndrome (closely related to tuberous sclerosis)3
*Occasionally coexists with:
  • renal cell carcinoma
  • collecting duct carcinoma
  • adenoma
  • oncocytoma

Metastases
Rare but retroperitoneal deposits or nodules in the lung and other organs. Hamartoma (loạn sản phôi)