Abstract
Angiomyolipomas (AMLs) are benign tumours containing smooth muscle, fat and abnormal blood vessels. They have recently been recognized as clonal neoplasms rather than hamartomas, with a significant proportion containing clonal chromosomal aberrations. An epithelioid variant of AML has been identified. The majority of conventional and epithelioid AMLs have an entirely benign course, although there is a risk of catastrophic haemorrhage. However, several cases of AMLs with malignant potential have been reported. The first described case was a patient with sarcomatous degeneration of a renal AML with pulmonary metastases. The tumour cells in such cases stain strongly for melanoma-associated markers, and are thought to be derived from perivascular epithelioid cells (PECs). Malignant epithelioid AML arising in the kidney is a distinct disease entity, separate from renal cell carcinoma (RCC). We report on a case of a patient with a malignant epithelioid AML of the kidney with an adjacent conventional AML. The ultrasound and computed tomography (CT) appearances of the tumour are described. The patient also had multiple hepatic lesions, with the typical imaging characteristics of haemangiomas.
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