[Aggressive fibromatosis in childhood]

Despite its benign microscopic appearance and nonmetastatic behavior, aggressive fibromatosis infiltrates neighboring tissues and has a considerably high recurrence rate (30%-60%). It is a rare lesion (0.03%-0.1% of all tumors) with an incidence of between two and four cases per million residents and per year. There is no agreement regarding the etiology of fibromatosis. Hormonal, traumatic and genetic influences have been described. It is a common manifestation in Gardener syndrome. The treatment of choice is broad surgical resection. When resection is incomplete or impossible, radiotherapy, chemotherapy or chemotherapy in combination with hyperthermia is the second choice of treatment. On the basis of five children observed, we discuss the etiologic factors, classification, clinical aspects and results of surgical and medical therapy.