A Case of Multicentric Infantile Myofibromatosis / 대한소아혈액종양학회지

ABSTRACT

Infantile myofibromatosis is one of the most common fibrous proliferation of infancy, characterized by the development of non-tender, firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and subcutaneous tissues. Three distinct subtypes are recognized-solitary, multicentric and generalized subtype. In solitary and multicentric subtypes, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. But the prognosis of generalized subtype is poor with 75% of patients dying within the first few months of life secondary to cardiopulmonary or gastrointestinal complications. We report a case of multicentric infantile myofibromatosis in a 4-year-old boy presenting with multiple, asymptomatic, fixed and protruding masses on the posterior occiput, shoulders, upper and lower back and right lateral chest wall. Over the subsequent 20 monthes, the child developed limitation of motion of back and shoulder with the repeated formation and disappearance of myofibromatosis of the skin and musculature, but without visceral involvement.