Sporadic unifocal infantile myofibromatosis involving the skull - Case report

Infantile myofibromatosis involving the skull is a benign disease if there is a solitary lesion. However, the multifocal form with skull involvement m ay portend a lethal course in the Ist year of life if there is involvement of the heart, lungs, or gastrointestinal tract. The authors report the case of a 3-year-old boy with an enlarging left parietal skull lesion that had been present since infancy. Increasing pain and the need to obtain tissue f or diagnosis led to resection of the lesion by means of a small craniectomy . Further evaluation revealed no other lesions. A distinctly rare disease i s presented, and the need for staging in children younger than 2 years of a ge is suggested to rule out cardiac, pulmonary, or gastrointestinal involve ment.