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Orthopaedics · Bone tumours: desmoid tumour
Sample itemA 28-year-old with familial adenomatous polyposis develops a firm, infiltrating mass in the abdominal wall after a laparotomy. Biopsy shows bland spindle cells with nuclear beta-catenin staining. What is the expected behaviour of this tumour?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Locally aggressive with frequent recurrence but no distant metastasis
Desmoid tumours (aggressive fibromatosis) are clonal fibroblastic proliferations linked to beta-catenin pathway mutations, often with Gardner syndrome or prior trauma or surgery. They infiltrate muscle and recur after excision, but do not metastasise. Because of high recurrence, wide excision, radiotherapy, systemic therapy or active surveillance are used.
Option by option
A. Malignant transformation to osteosarcoma
Desmoid tumours arise from fibroblasts and do not transform into bone-forming sarcomas.
B. Locally aggressive with frequent recurrence but no distant metastasis (correct)
Correct. They infiltrate locally and recur, but they do not metastasise.
C. Early haematogenous spread to the lungs
Lung metastases are a feature of sarcomas, not of desmoid tumours.
D. Spontaneous regression in all cases
Some stabilise or regress, but many progress and recur, so regression is not guaranteed.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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