TSC Angiomyolipoma Fat-Containing Mass — ESENeph MCQ

Instant feedback + full explanation. One question, done properly.

ModerateChronic Kidney DiseaseTSC Angiomyolipoma Fat-Containing MassESENeph

A 42-year-old man presents with severe loin pain, gross haematuria, and a flank mass. CT shows a large heterogeneous renal mass with areas of fat density (macroscopic fat). He has facial angiofibromas, periungual fibromas, and a history of epilepsy. The mass is 6 cm. What is the most likely renal lesion?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: D — Angiomyolipoma

The clinical triad of facial angiofibromas, periungual fibromas, and epilepsy is diagnostic of tuberous sclerosis complex (TSC). The renal mass containing macroscopic fat on CT is characteristic of angiomyolipoma (AML) — benign hamartomas composed of blood vessels, smooth muscle, and fat. AMLs occur in ~80% of TSC patients, are often bilateral and multifocal, and carry haemorrhage risk when >4 cm (from intra-tumoural aneurysms). The 6 cm size exceeds the intervention threshold. First-line treatment is everolimus (mTOR inhibitor) to shrink the lesion. Selective embolisation is reserved for acute haemorrhage. Nephron-sparing surgery is a last resort. The presence of macroscopic fat on CT is virtually pathognomonic for AML.

Reference: Bissler et al 2013 – EXIST-2; European TSC Consensus 2021; NICE 2023 – TSC