Brachial Plexus Desmoid Tumor: Care for Functional Preservation

Area of Science:

• Oncology
• Surgical Pathology
• Genetics

Background:

• Desmoid tumors are rare, locally invasive neoplasms of mesenchymal origin.
• While often sporadic, they can be associated with germline adenomatous polyposis coli mutations.
• Common sites include the abdomen, thorax, extremities, and head/neck, posing treatment challenges due to high recurrence rates.

Observation:

• An 18-year-old female presented with a desmoid tumor involving the brachial plexus.
• Surgical intervention focused on non-oncological resection for functional preservation.
• Residual tumor was identified post-resection.

Findings:

• The desmoid tumor infiltrated the brachial plexus, necessitating a complex surgical approach.
• Despite achieving negative margins, residual disease was present.
• Treatment for residual disease involved a β-catenin inhibitor with serial MRI monitoring.

Implications:

• This case underscores the challenges of managing desmoid tumors, particularly in critical neurovascular areas like the brachial plexus.
• Functional preservation is a key consideration in surgical planning for such tumors.
• Targeted therapies, such as β-catenin inhibitors, show promise in managing residual or recurrent desmoid tumors.