Function Preserving Surgery for Brachial Plexus Fibromatosis with Mediastinal Extension

Abstract

Desmoid tumors or fibromatosis is a benign but locally aggressive and infiltrative disease with origin attributed to musculoaponeurotic fibrous tissue. Despite being a locally aggressive tumor, infrequent mitosis, absence of necrosis, and absence of metastasis reconfirm its benign nature.[1] Fibromatosis in general is a rare entity comprising 0.03% of all tumors in humans.[2] Most cases of fibromatosis are intra-abdominal and extra-abdominal fibromatosis is less common. Head and neck are also an uncommon locations making up to 7%–25% of extra-abdominal desmoid tumors.[3] The precarious location in the head and neck combined with aggressive nature of these tumors often causes involvement of vital neurovascular structures ranging from involvement of the frontal bone and palate to extension into the mediastinum being reported.[1,4] In this article, we describe a case of neck fibromatosis involving the brachial plexus in a 24-year-old female that was associated with motor and sensory deficit in the ipsilateral upper limb. We discuss the operative technique and associated nuances of the case, highlighting the multidisciplinary surgical approach to achieve maximal safe resection while preserving neurological function.