Infantile myofibromathosis of the maxilla. A case report

Infantile myofibromatosis (IM) is a rare benign tumor in children. It was first described as congenital fibrosarcoma. Later, sporadic cases were discussed, and in 1981, 61 cases were examined and named as IM. Three different forms were described: solitary, multicentric and visceral IM.1 Three quarters of soft tissue tumours in children and adolescents are benign, 95 % of them are fibromatosis, 80 % of fibromatosis are IM and 19 % aggressive desmoid fibromatosis.2 The most frequent type is solitary IM. One third of these occur are in the head and neck. Its aetiology is not known. First, it was believed that original cells are fibroblasts; but with the presence of desmins receptors in IM, this belief proved to be false. In fact, the original cells of IM growth are smooth muscular cells.3 Genetic predisposition is likely since solitary form is twice as frequent in males than in females. Clinically, IM is expressed by slowly growing painless firm solitary or multicentric nodes in the soft tissue, bones or visceral organs. Half of the cases develop in the dermis or subdermally.4 Their size is few millimetres to few centimetres. Half of the cases are congenital, 90 % of all cases develop in first Radiol Oncol 2002; 36(1): 47-51.