Epithelioid angiomyolipoma of kidney

Last revised by Liz Silverstone on 26 Oct 2021

Epithelioid angiomyolipomas (EAML) are rare variants of the more common renal angiomyolipoma. They have malignant potential.

Like more common renal angiomyolipomas, EAMLs are considered perivascular epithelioid cell tumors (PEComas). EAMLs were regarded as a separate renal mass entity by the WHO in 2016.

Epithelioid angiomyolipomas do not have a specific appearance. Some features that have been associated with them:

  • hyper-attenuating mass with variable enhancement 3
  • T2: hypointense
  • IP/OOP imaging: signal dropout of microscopic fat content on OOP
  • DWI/ADC: hyperintense on DWI
  • T1 C+: marked hypervascularity with rapid washout

The imaging appearance is similar to renal cell carcinoma and AML with minimal fat.

The diagnosis can be considered if a renal mass has small foci of macroscopic fat without calcification, which would be atypical for renal cell carcinoma. Renal biopsy would be needed for confirmation, however.

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