[Clinical analyses of bilateral renal angiomyolipomas associated with tuberous sclerosis complex]

To explore the clinical characteristics, diagnosis, treatment and outcomes of renal angiomyolipoma (RAML) associated with tuberous sclerosis complex (TSC).

The clinical data were analyzed for 10 cases of renal angiomyolipomas associated with tuberous sclerosis. There were 4 males and 6 females with a mean age of 23.5 (9-44) years.Five patients were admitted for flank pain or hematuria.RAML was found by TSC associated imaging examinations for those with extra renal organ involvement.

Bilateral multiple renal angiomyolipomas were all confirmed on imaging tests and consistent with clinical TSC. The maximal diameter of RAMLs was from 2 to 15(7.3 ± 3.5) cm.Six patients of tumor hemorrhage had an average maximal diameter of 9.2 cm.Selective arterial embolization (SAE) (n = 4) and homeostasis, anti inflammation and immobilization therapy (n = 2) were performed.Four asymptomatic cases accepted close observation. During a follow-up period of 9-116 months, one of 4 SAE cases underwent secondary SAE due to rebleeding 12 months later while there was no recurrence of flank pain in other 3 cases. The mean enlargement of maximal diameter was 1.5 cm/year and serum creatinine elevated by 3.1 µmol/L/year.Six cases on conservative medical therapy or under observation had a mean enlargement of maximal diameter of 0.7 cm/year and serum creatinine elevated by 2.2 µmol/L/year. Three cases remained asymptomatic, one died of respiratory failure from TSC-associated pulmonary lymphangioleiomyomatosis, two had flank pain at 16 and 40 months respectively, one underwent secondary SAE and another received rapamycin with marked tumor regression.

Bilateral multiple RAMLs occur in most TSC patients. After excluding malignancy, SAE becomes an important choice for its mini-invasiveness, efficacy and chances of re-embolization. And rapamycin provides ameliorative treatment for TSC patients.