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TSC Renal Monitoring — RACP Paediatrics MCQ

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HardNeurology & DevelopmentTSC Renal MonitoringRACP Paediatrics

A 13-year-old with tuberous sclerosis complex has bilateral renal angiomyolipomas on surveillance MRI. One lesion has enlarged from 2.5 cm to 3.4 cm over 18 months. There is no aneurysmal bleeding, pain or haemodynamic instability. What is the preferred disease-directed approach?

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Reveal the answer and explanation

Correct answer: CDiscuss systemic mTOR-inhibitor therapy through the established TSC service

Explanation lettering: C = shown as A · A = shown as B · D = shown as C · B = shown as D

D is correct because an asymptomatic but growing TSC-associated angiomyolipoma larger than 3 cm is generally managed first with an mTOR inhibitor, balancing renal and multisystem benefits against toxicity. A uses an interval longer than the recommended lifelong 1–3-year imaging schedule and ignores growth. B sacrifices nephrons in a bilateral lifelong disease. C is important for acute haemorrhage or selected anatomy but is not routine first-line therapy for this stable presentation. E has no disease-modifying role. The international TSC consensus is used by Australian tertiary services and its renal recommendation is jurisdiction-neutral.

Reference: Healthdirect Australia, tuberous sclerosis: https://www.healthdirect.gov.au/tuberous-sclerosis; International Tuberous Sclerosis Complex Consensus Group, updated surveillance and management recommendations: https://pubmed.ncbi.nlm.nih.gov/34399110/