skip to main content

VHL Syndrome — SCE Medical Oncology MCQ

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

HardCancer GeneticsVHL SyndromeSCE Medical Oncology

A 38-year-old with von Hippel-Lindau syndrome has several bilateral enhancing renal lesions. The largest is 2.7 cm, serial MRI shows slow growth, renal function is normal and there are no aggressive clinical features. What is the preferred current approach?

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

Reveal the answer and explanation

Correct answer: D — Continue specialist MRI surveillance until the largest lesion approaches 3 cm, then plan nephron-sparing treatment

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

E is correct. NICE NG256 supports active surveillance for lesions below 3 cm in a heritable syndrome not associated with highly aggressive RCC, coordinated through the uro-oncology and syndrome MDTs. VHL patients may develop repeated bilateral tumours, so preserving renal parenchyma is crucial when intervention becomes necessary. Multifocality does not mandate bilateral nephrectomy, systemic immunotherapy is not treatment for small localised lesions, and repeated biopsy of every lesion adds morbidity without solving management. Belzutifan has a defined specialist role when VHL-associated tumours need treatment and surgery is unsuitable, but it is not automatic at 2.7 cm.

Reference: NICE NG256 hereditary renal-cancer management: https://www.nice.org.uk/guidance/ng256/chapter/Managing-renal-cell-carcinoma-in-people-with-a-heritable-renal-cell-carcinoma-predisposition-syndrome