CURE OF INFANTILE MYOFIBROMATOSIS WITH SEVERE RESPIRATORY COMPLICATIONS WITHOUT ANTITUMOR THERAPY

The prognosis of infantile myofibromatosis (IMF) depends on the organs involved: the prognosis is very poor if vital viscera are affected, b ut excellent if there is no visceral involvement. We report the case o f a boy presenting with a pathological fracture at the age of 6 weeks. Progressive osteolytic lesions in the whole skeleton until the age of 8 months led to respiratory failure due to a softened thoracic wall r equiring mechanical ventilation for 11 months. No pulmonary, laryngeal or other visceral involvement was found. In spite of the rapidly prog ressing disease and serious complications only supportive therapy was given. The lesions subsided gradually leaving slight deformities but n ormal function. At the age of 3.5 years the boy has an excellent quali ty of life. Conclusion This case illustrates that even in progressing, complicated multifocal infantile myofibromatosis (without visceral in volvement); the lesions can resolve without antitumour treatment if hi gh quality intensive care is supplemented.