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This case study describes a 50-year-old man with a desmoid tumor of upper extremity, which had to be resected concomitant with the left arm amputation at middle third of the humerus. The course was initially stable, but evolved with aggressive infiltration of local soft tissues. During the watchful waiting time, three biopsies were done before establishing the diagnosis. Plain X-ray, magnetic resonance and positron emission tomography were the exams utilized. The authors emphasize diagnostic and management challenges involving this rare condition. Case reports can stimulate further studies to get additional knowledge about this rare entity.
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