Cure of infantile myofibromatosis with severe respiratory complications without antitumour therapy

• Published in: European journal of pediatrics Vol. 156; no. 11; pp. 841 - 844
• Main Authors: ZELLER, B, STORM-MATHISEN, I, SMEVIK, B, SUND, S, DANIELSEN, K, LIE, S. O
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01.11.1997; Berlin Springer Nature B.V
• Subjects: Biological and medical sciences; Bone Neoplasms - complications; Bone Neoplasms - diagnosis; Bone Neoplasms - therapy; Fractures, Spontaneous - etiology; Humans; Infant; Male; Medical sciences; Myofibromatosis - complications; Myofibromatosis - diagnosis; Myofibromatosis - therapy; Remission, Spontaneous; Respiration, Artificial; Respiratory Insufficiency - etiology; Respiratory Insufficiency - therapy; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Thorax; Human; Case study; Infant; Multiple; Benign neoplasm; Fibroblast
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s004310050725

The prognosis of infantile myofibromatosis (IMF) depends on the organs involved: the prognosis is very poor if vital viscera are affected, but excellent if there is no visceral involvement. We report the case of 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 requiring mechanical ventilation for 11 months. No pulmonary, laryngeal or other visceral involvement was found. In spite of the rapidly progressing disease and serious complications only supportive therapy was given. The lesions subsided gradually leaving slight deformities but normal function. At the age of 3.5 years the boy has an excellent quality of life. This case illustrates that even in progressing, complicated multifocal infantile myofibromatosis (without visceral involvement) the lesions can resolve without antitumour treatment if high quality intensive care is supplemented.