Association between the growth rate of renal cysts/angiomyolipomas and age in the patients with tuberous sclerosis complex

PURPOSE: Renal manifestations of tuberous sclerosis complex (TSC) occur with a high frequency and a wide range of severity. The onset and complications of each affected organ depend on the age. This study aimed to investigate the associations between comorbidities, frequency, and size of cysts/angiomyolipomas of TSC and the patients in the different ages.METHODS: We performed a systematic evaluation of patients with TSC at integrated clinics for TSC. The patients were diagnosed with TSC according to Roach's clinical diagnostic criteria. All the patients underwent a systematic evaluation with a medical history review, recording of cutaneous manifestations, magnetic resonance imaging of the brain, chest computed tomography scan, and cardiac echography. Each patient was routinely evaluated with renal magnetic resonance imaging.RESULTS: Of the 57 patients with TSC, renal lesions or manifestations were observed in 38.5 % at preschool-age group (<6 years) that increased to 75.0, 85.7, and 100 % in school-age group (6-18 years), young adulthood group (18-30 years), and middle-aged group >30 years, respectively. Renal complications or interventions were found in 72.7 % of patients >30 years. There was a strongly positive association between the size of renal lesions and age (p < 0.001). Comorbidities of TSC were significantly correlated with pulmonary lymphangiomyomatosis (p < 0.001) and seizure remission (p = 0.018).CONCLUSIONS: The results indicate a positive association of progressively growing renal lesions in patients with TSC with increasing age. It is mandatory for clinicians that monitor the high-risk patients to minimize and prevent renal complications in the future.