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