870 Desmoid tumors (aggressive fibromatosis): Retrospective study

Abstract

Although benign, desmoid tumors are locally aggressive neoplasms which infiltrate adjacent tissues, resulting in a high incidence of local recurrence after conservative resection. Between 1982 and 1993, 10 female and 5 male patients with histologically confirmed desmoid tumors were referred to Instituto Portugues de Oncologia—Porto. Age ranged from 12 to 47 years, with 2 newborn patients. Sites of disease included head and neck (n = 5), shoulder girdle (n = 3), chest wall (n = 2), abdomen (n = 2), extremities (n = 2) and back (n = 1). Patients were treated with surgery alone (n = 3) or surgery plus radiation (n = 12). Ten patients underwent radiation therapy for uncertain, positive margins or subtotal resection and 2 received planned postoperative radiation (microscopically negative margins), the majority being treated to a tumor dose of 40–70 Gy. With a medium follow up of 4.5 years, 14/15 patients are without evidence of disease and one died with progressive multicentric disease. In the irradiated group, 2 patients with infield recurrence and another with marginal recurrence were successfully treated with surgery. In summary, we believe that moderate doses of radiation can improve local control rates minimal long-term effects.