Giant Desmoid-Type Fibromatosis of the Neck: A Very Rare Infiltrating Tumor

Summary

A 44-year-old woman was referred to the department of head and neck surgery for a 12-month history of decubitus dyspnea in the context of a painless mass of the right side of the neck, blocking the homolateral shoulder joint. Medical history reported the realization of a biopsy of the mass 4 months ago in Chechnya with a diagnosis of rhabdomyoma of the right side of the neck. The patient had never underwent other surgical procedures. Since then, the size of the mass continued to expand rapidly, leading to a recent decubitus dyspnea. The ear, nose, and throat examination revealed a very painful mass located in the right side of the posterior cervical area next to the cervical scar of the previous biopsy. Magnetic resonance imaging (MRI) showed an extensive tumor of the neck basis measuring 20 × 15 cm and infiltrating the nerve roots of the brachial plexus, the scalene muscles, the right carotid, the right phrenic nerve, and the adjacent adipose tissue. The chest X-ray found a right-side elevation of the right diaphragmatic cupola highlighting the paresis of the right side of the diaphragm. A fine-needle aspiration biopsy was performed but the result was noncontributory. Regarding the risk of malignancy, a debulking of the mass was performed with a frozen examination that could not found the histopathological nature of the lesion. Vascular and nerve structures have been conserved and there was no postoperative defect. The postsurgery histopathological examination of the mass revealed a desmoid-type fibromatosis of the neck. The reconstruction was made with a right pectoralis major musculomyocutaneous flap. Following the discussion of the oncological multidisciplinary group, patient received complementary radiation for the residual tissue. The 4-year follow-up was unremarkable.