Desmoid tumors of the head and neck - A clinical study of a rare entity

Background. Desmoid tumors are are, nonmalignant neoplasms that show locall y aggressive growth but lack the potential to metastasize. Common anatomic sites include the extremity, abdominal wall, and mesentery. Little is repor ted about clinical features and outcome of desmoid tumors of the head and n eck. Methods. Twenty-one patients with desmoid tumors of the head and neck treat ed at MSKCC between July 1982 and June 1999 were identified from our inpati ent tumor database. All patients underwent surgical resection and were pros pectively followed. Clinicopathologic features and treatment modalities wer e evaluated. Results. Patient age at diagnosis was 22 to 76 years, with a female-male ra tio of 16:5. Most tumors were located in the supraclavicular fossa or the m uscles of the neck (n = 19). Patients most commonly had a painless mass (n = 13) or neurologic symptoms including pain or neurologic deficit (n = 8). Extent of resection was limited to the tumor in nine patients. In the other 12, structures including the accessory or phrenic nerve, parts of the brac hial plexus, or bony structures were resected. Persistent neurologic or fun ctional deficits after surgery were noted in 12 patients. Five of 21 patien ts had recurrences (24%). Eleven patients received radiation therapy. In th is small population, no obvious benefit for patients receiving radiation vs patients who were not irradiated, regardless of their surgical margin stat us, was seen. Treatment for recurrence was re-resection or re-resection plu s radiation. Eighteen patients remained free of disease after treatment, tw o patient have stable disease and one died of other causes. No patients die d of their disease. Conclusions. Desmoid tumors of the head and neck are rare, fibrous neoplasm s with a good prognosis but significant morbidity. Function-preserving surg ery should be a primary goal to minimize morbidity. in this small group of patients, the benefits of radiation therapy in patients with positive margi ns could not be clearly demonstrated and should be balanced against radiati on-related morbidity. (C) 2000 John Wiley & Sons, Inc.