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Desmoid Tumour — SCE Medical Oncology MCQ

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HardSarcomaDesmoid TumourSCE Medical Oncology

A 45-year-old woman presents with a desmoid tumour (aggressive fibromatosis) in the abdominal wall, 8 cm in diameter. It is not causing functional impairment. Biopsy confirms the diagnosis with CTNNB1 (β-catenin) mutation. What is the recommended initial management?

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Correct answer: CActive surveillance (watch and wait)

Current guidelines (ESMO 2024) recommend active surveillance as the initial approach for most desmoid tumours, as approximately 20-30% undergo spontaneous regression and many remain stable. Surgery carries high recurrence rates (25-60%). Systemic therapy (sorafenib, gamma-secretase inhibitors, low-dose chemotherapy) is reserved for progressive or symptomatic tumours. The DESMOID trial and consensus guidelines support this shift away from primary surgery.

Reference: ESMO 2024 Desmoid Tumour Guidelines; Kasper et al Ann Oncol 2017