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Fibrotic hypersensitivity pneumonitis — SCE Respiratory MCQ

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HardILDFibrotic hypersensitivity pneumonitisSCE Respiratory

A very large solitary fibrous tumour of the pleura is accompanied by recurrent fasting hypoglycaemia, suppressed insulin and suppressed C-peptide. Which tumour product causes this paraneoplastic syndrome?

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Correct answer: E — High-molecular-weight IGF-II

Doege-Potter syndrome is non-islet-cell tumour hypoglycaemia associated with a solitary fibrous tumour, classically a large pleural lesion. Incompletely processed high-molecular-weight insulin-like growth factor II increases peripheral glucose uptake and suppresses insulin, C-peptide, growth hormone and IGF-I. Definitive tumour resection usually corrects the hypoglycaemia.

Reference: Clinical review of solitary fibrous pleural tumours and Doege-Potter syndrome (Published 2015): https://pmc.ncbi.nlm.nih.gov/articles/PMC4635284/; Review of solitary fibrous pleural tumours and Doege-Potter syndrome (Published 2023): https://pmc.ncbi.nlm.nih.gov/articles/PMC10453165/