Renal and hepatic angiomyolipomas in tuberous sclerosis

Case contributed by Dr Jan Frank Gerstenmaier

Diagnosis almost certain

Presentation

Screening examination. Known tuberous sclerosis

Patient Data

Age: 35 years

Gender: Female

From the case: Renal and hepatic angiomyolipomas in tuberous sclerosis

MRI

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Coronal T2

Both kidneys contain numerous (in excess of 10 each side) fat signal lesions ranging in size between pinpoint and up to 5 mm on the left, and 6 mm on the right, consistent with renal angiomyolipomas. No evidence of cysts or a solid enhancing renal mass, The liver also contains several small foci of subcentimetre fat signal lesions in keeping with hepatic angiomyolipomas. No retroperitoneal cysts. No free fluid or lymphadenopathy. Pancreas, adrenal glands, spleen are normal.

Conclusion: Numerous subcentimetre renal as well as hepatic angiomyolipomas confirmed. No renal or retroperitoneal cysts.

Case Discussion

20% of patients with renal angiomyolipomas have tuberous sclerosis. 55-75% of patients with tuberous sclerosis have renal angiomyolipomas.

The association between tuberous sclerosis and hepatic angiomyolipomas is less strong - only 6% of hepatic angiomyolipomas have tuberous sclerosis, but the liver is the second most common organ involved.