Aggressive fibromatosis of the head and neck：case report and literature review

Abstract

Objective To evaluate the clinical manifestations, pathological features, treatment methods and prognosis of aggressive fibromatosis of the head and neck. Methods One patient with aggressive fibromatosis of the neck was analyzed, and the relevant literature was reviewed. Results Head and neck lesions account for approximately 12 to 15% of aggressive fibromatosis, which is a rare type of borderline tumor that is commonly characterized by a hard texture, painlessness (but occasionally with pain), hidden growth and poor mobility. Such tumors can result in facial deformity and invasion of the skull base or main nerves, and blood vessels and can compress the airway. MRI is the preferred method for preoperatively determining the size and location of the lesion. The characteristic low T1 and T2 signals of collagen fiber are helpful for the diagnosis of the tumor. The disease has clear pathological features, with tumors consisting of long spindle fibroblasts and myofibroblasts arranged in parallel fascicles with various levels of collagen formation. The tumor cells exhibit the characteristics of infiltrative growth, ill ⁃ defined cell membranes and variable amounts of cytoplasm. Pathologic mitosis and atypia are not seen. Characteristic immunohistochemical features include expression of Vim(+), HHF⁃35(+), CD34(⁃), S⁃100(⁃) and Ki⁃67(+). The disease is locally invasive, and patients may relapse easily, but distant metastases are not observed. The primary treatment is surgical resection. Chemotherapy, hormone therapy and biological treatments have auxiliary functions in the treatment of these tumors. Conclusion Aggressive fibromatosis of the head and neck has no specific clinical features; the diagnosis depends primarily on pathological examination. The main treatment is radical surgical resection. Radiotherapy, chemotherapy and biological treatment can be used in combination with surgery in cases of incomplete resection or recurrence.

Keywords

• Head and neck，
• Neoplasm，
• Fibromatosis，
• Critical tumor，
• Invasiveness，
• Recurrence，