Case report of recurrent fibromatosis with laryngeal involvement: Treatment based on network analyses of NGS data

Summary

Aggressive fibromatosis (AF) is a rare, benign neoplasm originating from musculoaponeurotic stromal structures characterized by aggressive growth and infiltration of local tissues. To date, only six previous cases of AF involving the larynx have been reported. The present case was that of a 70-year-old female patient with a 5-year history of hoarseness and an enlarging neck mass consistent with aggressive recurrent fibromatosis. MRI displayed a large, solid mass arising from the left anterior cervical space displacing the trachea and upper airway to the right. At one year after the initial radical resection, the patient presented with recurrence. A second radical excision was performed not including a laryngectomy. Radiation therapy was considered for possible local treatment to prevent subsequent tumor recurrence. According to the literature, AF has recurrence rates of up to 40-70% within 18 months. There is only sparse literature to guide treatment. Using the mutations detected in the patient's AF tissue, an Ingenuity Pathway Analysis (IPA) was used to guide treatment of the recurrence. In the present case, the IPA analysis indicated the use of pazopanib to treat the patient's cancer. In general, surgery appears to be the treatment of choice for head and neck AF, but the management of recurrence is controversial.