Management of renal angiomyolipoma in complex clinical situations

Renal angiomyolipoma (AML) is associated with complex clinical situations s uch as tumour in a solitary kidney, bilateral, large or multicentric tumour s 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 h ad TS, 5 had multiple and bilateral tumours, 1 patient was pregnant and 1 h ad a solitary functioning kidney. With the newer imaging modalities correct diagnosis was possible in 12 cases and renal cell carcinoma (RCC) was susp ected in 3 cases. Selective angioembolization (SAE) was done in 3 patients, which successfully controlled bleeding in all. Nephron-sparing surgery (NS S) 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 kidne y. Three patients with multiple and bilateral tumours were chosen for conse rvative treatment and none developed recurrence of bleeding on strict follo w-up. In a pregnant patient, bleeding was successfully controlled with angi oembolization. However, 1 patient with a solitary functioning kidney with l arge-sized tumour (20 x 18 cm) underwent NSS. In conclusion, the basis of m anagement 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 t he ideal treatment, if feasible. The patients in the TS group are usually m ore complicated and require life-long follow-up after initial management wi th NSS or SAE. Pregnant AML patients can be safely managed with SAE. Conser vative treatment without any intervention and regular follow-up may be more helpful in some patients with multiple, bilateral extensive tumours. Copyr ight (C) 2001 S. Karger AG, Basel.