Renal angiomyolipoma (AML) is associated with complex clinical situations such as tumour in a solitary kidney, bilateral, large or multicentric tumours or those associated with tuberous sclerosis (TS) or pregnancy. Management in these situations may be challenging. Fifteen patients (20 kidneys) were admitted with symptomatic AML over last 10 years. Eleven patients had one or the other complicating factor. Ten patients had a tumour of >10 cm, 4 had TS, 5 had multiple and bilateral tumours, 1 patient was pregnant and 1 had a solitary functioning kidney. With the newer imaging modalities correct diagnosis was possible in 12 cases and renal cell carcinoma (RCC) was suspected in 3 cases. Selective angioembolization (SAE) was done in 3 patients, which successfully controlled bleeding in all. Nephron-sparing surgery (NSS) was performed in 5 patients. Total nephrectomy was done in 4 cases, in 3 due to suspicion of RCC and in 1 due to extensive involvement of the kidney. Three patients with multiple and bilateral tumours were chosen for conservative treatment and none developed recurrence of bleeding on strict follow-up. In a pregnant patient, bleeding was successfully controlled with angioembolization. However, 1 patient with a solitary functioning kidney with large-sized tumour (20 × 18 cm) underwent NSS. In conclusion, the basis of management of AML is preservation of renal tissue, which can be effectively achieved with SAE or NSS. In a solitary functioning kidney, NSS or SAE is the ideal treatment, if feasible. The patients in the TS group are usually more complicated and require life-long follow-up after initial management with NSS or SAE. Pregnant AML patients can be safely managed with SAE. Conservative treatment without any intervention and regular follow-up may be more helpful in some patients with multiple, bilateral extensive tumours.

Keywords:
Angiomyolipoma, Kidney, Nephrectomy, Nephron-sparing surgery, Angioembolization, Tuberous sclerosis
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© 2001 S. Karger AG, Basel
2001
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