Adolescent testicular microlithiasis: A case-based, multinational survey of clinical management practices.

INTRODUCTION: Testicular microlithiasis (TM) is a condition characterized by calcium deposits within the testis, usually detected incidentally during ultrasonography of the scrotum. TM has been associated with the presence of, and possibly the development of, testicular malignancy. Our aim was to document international clinical management practices for TM and to analyze what factors and perception of risk influence conservative versus active management and follow-up. METHODS: European Society for Paediatric Urology (ESPU) and Society for Pediatric Urology (SPU) members were invited to complete an online case-based survey of clinical management practices of TM. Eight cases had a single variable changed each time (classic versus limited TM, unilateral versus bilateral, prior cryptorchidism versus no cryptorchidism) to ascertain the provider's perception of risk. The respondents completed multiple choice questions on initial management, follow-up plan, length and interval of follow-up. Multivariate logistic regression was performed to determine factors associated with decisions on management and follow-up. RESULTS: There were 265 respondents to the survey from 35 countries (Table). Median time in practice was 13 years. Factors that were significantly associated with more aggressive initial management (more than counseling on self-examination) included: not yet in independent practice, low volume TM cases per year, those practicing pediatric and adult urology, classic appearance of TM and cryptorchidism. Factors that were significantly associated with urologist follow-up and active investigation included: European practitioners, low TM case volume per year, those practicing both pediatric urology and pediatric surgery, classic TM appearance and a case history of cryptorchidism. Interval and length of follow-up was wide-ranging, with most respondents favoring annual follow-up. CONCLUSION: Management of TM varies and a mix of surgeon and case factors significantly influences management strategies. This baseline understanding of the lack of systematic management suggests the need for the development of consensus guidelines and prospective study.

Pediatric Renal Angiomyolipomas in Tuberous Sclerosis Complex.

PURPOSE: Tuberous sclerosis complex is a genetic disorder characterized by the growth of hamartomas in multiple organs. Up to 80% of patients with tuberous sclerosis complex will have at least 1 angiomyolipoma in their lifetime. We describe the incidence and natural history of angiomyolipoma in a pediatric tuberous sclerosis complex population and analyze tumor growth to determine optimal renal imaging intervals in an effort to improve counseling, treatment and followup. MATERIALS AND METHODS: We performed a retrospective chart review of all patients with tuberous sclerosis complex from 2004 to 2014. Patients were included if they had a clinical or genetic diagnosis of tuberous sclerosis complex and had undergone at least 1 renal imaging study. RESULTS: A total of 145 patients were analyzed. Median age was 14 years (range 0 to 28). Overall incidence of angiomyolipoma was 50.3%. Median age at first angiomyolipoma detection was 11 years (range 2 to 26). Median yearly angiomyolipoma growth rate stratified by age at first detection was 0.0 mm for patients 0 to 6 years old, 0.9 mm for those 7 to 11 years old, 2.5 mm for those 12 to 16 years old and 1.8 mm for those 17 years old or older. Median yearly angiomyolipoma growth rate stratified by tumor size at first detection was 0.1 mm for tumors 0.6 to 0.9 cm, 1.8 mm for those 1.0 to 1.9 cm and 4.3 mm for those 2.0 to 2.9 cm. A total of 35 patients (24.1%) received mTOR (mammalian target of rapamycin) inhibitors. Eight patients underwent a total of 13 surgical interventions, of whom 2 had previously been treated with mTOR inhibitors. Median patient age at surgical intervention was 18.0 years and median angiomyolipoma size was 5.0 cm. CONCLUSIONS: Angiomyolipoma growth in children with tuberous sclerosis complex can be rapid and unpredictable. We recommend yearly renal ultrasound in all patients with tuberous sclerosis complex, with consideration of magnetic resonance imaging in those at risk for rapid growth and future intervention (ie those older than 11 years and/or those with renal angiomyolipomas larger than 2 cm).