Desmoid tumor of the head and neck

BACKGROUND: [corrected] Desmoid tumors are rare benign tumors but have a tendency toward local recurrence after resection because of their infiltrative growth and frequent entrapment of vital structures in the head and neck region. We report 24 desmoid tumors of the head and neck and propose a reasonable approach in the management of such cases. METHODS: Twenty-four patients (9 male and 15 female; median age, 33 years; range, 0-66 years) with a desmoid tumor of the head and neck (neck, 15 patients; head, 9 patients) treated from 1990 to 2004 were retrospectively analyzed. The size ranged from 0.5 to 13 cm in diameter (mean, 3.6 cm). In the neck, 8 tumors were around the superficial layer of deep cervical fascia, whereas 4 tumors of the neck involved the prevertebral fascia and 2 involved brachial plexus. RESULTS: Twenty patients received complete resection of the tumor, but the section margin was positive in 8 patients, of which 6 patients remained free of disease in a period of 13 to 105 months. Three patients, including 2 with positive section margin and 1 with negative margin, developed recurrences, which were successfully removed again. Two patients underwent partial resection of the tumor because of brachial plexus involvement. One of them achieved regression after postoperative radiotherapy and the other had spontaneous regression. The hypopharygneal tumor in a newborn had spontaneously complete regression, and tracheostomy was closed at the age of 6 years. One patient remained with stable disease for 14 months after biopsy of the tumor without excision. CONCLUSION: The overall prognosis is still good despite frequent incomplete resection. Surgical resection of the tumor with close observation is suggested even if the section margin is positive. If a desmoid tumor cannot be removed grossly, regression or arrested growth of the remaining tumor is expected. Radiotherapy might be reserved for a growing tumor.