Doege-Potter Syndrome — SCE Medical Oncology MCQ

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HardSarcomaDoege-Potter SyndromeSCE Medical Oncology

A 50-year-old woman with a solitary fibrous tumour (SFT) of the pleura develops recurrent hypoglycaemia. Insulin levels are appropriately suppressed. IGF-II is markedly elevated. What paraneoplastic syndrome does this represent?

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Correct answer: B — Doege-Potter syndrome

Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumours (previously called hemangiopericytomas). Tumours secrete high-molecular-weight ('big') IGF-II that activates insulin receptors and IGF-1 receptors, causing severe refractory hypoglycaemia. Diagnostic features: hypoglycaemia with suppressed insulin, C-peptide and GH, and elevated IGF-II:IGF-I ratio. Treatment: surgical resection (curative), glucocorticoids (suppress IGF-II production), and IV glucose for acute episodes.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/cancer