Desmoid-Type Fibromatosis of the Neck with Brachial Plexus Involvement: A Rare Case Report

Tóm tắt

Introduction: Desmoid-type fibromatosis (DTF) is a rare soft tissue tumor arising from musculoaponeurotic connective tissue, characterized by locally aggressive growth and a high rate of local recurrence despite the absence of metastatic potential.

Case summary: We report a case of a 19-year-old female patient presenting with a right cervical–supraclavicular mass associated with progressive upper limb numbness. Magnetic resonance imaging showed a large soft tissue mass infiltrating adjacent muscles and surrounding the bundles of the brachial plexus. Ultrasound-guided needle biopsy confirmed the diagnosis of desmoid-type fibromatosis. The patient underwent surgical dissection with maximal tumor resection combined with decompression and preservation of the brachial plexus. Postoperative histopathological results were consistent with DTF, with immunohistochemical staining for β-catenin positive. The postoperative course was favorable, with marked improvement in neurological symptoms and preservation of upper limb function. Although postoperative magnetic resonance imaging revealed residual lesions, the patient was selected for periodic follow-up instead of radiotherapy due to the risk of long-term radiation-related complications in a young patient.

Conclusion: This case demonstrates that maximal tumor resection combined with preservation of neural structures may be an appropriate treatment strategy for desmoid-type fibromatosis of the cervical–supraclavicular region involving the brachial plexus.