Function-preserving surgery in sporadic desmoid-type fibromatosis of brachial plexus.

Abstract

Desmoid tumours of the brachial plexus are rare locally infiltrative aggressive, monoclonal, fibroblastic proliferations characterized by a variable and often unpredictable clinical course. Only 21 patients have been reported in the literature. We add another one, and report function-preserving surgery in a 34-year-old man with a desmoid tumour of the brachial plexus. The patient presented with paraesthesia and gradually progressive distal muscle weakness in the left upper limb. Electrodiagnostic studies revealed preganglionic changes in segments C8-D1. Contrast-enhanced magnetic resonance imaging showed an enhancing mass with irregular margins in the left paravertebral region encasing the subclavian artery, pre- and post-ganglionic C6-D1 nerve roots and trunks of the brachial plexus. Using an anterior transclavicular approach the tumour was decompressed, which led to a major improvement in paraesthesia and partial motor recovery. He was doing well at 6 months of follow-up. Histopathological examination showed findings consistent with desmoid tumour. A tailored multidisciplinary surgical approach, with the aim to preserve function over radiological clearance, is an acceptable treatment strategy in preserving patient's quality of life for such infiltrating desmoid tumours encasing the brachial plexus. Following surgery, observation and close radiological surveillance offer an optimal strategy without jeopardizing the quality of life.