Lipofibromatosis is a rare, benign fibrofatty tumor of childhood. Since the tumor was first characterized in 2000, only a few additional cases have been reported. The classic presentation of lipofibromatosis is a slow-growing mass arising in a distal extremity, with boys more commonly affected than girls. We report a 16-month-old girl who presented with a lipofibromatosis in her left scapular region that increased 30% in size during a 6-week period. Although previous case reports of lipofibromatosis have emphasized immunohistological findings, this paper focuses on imaging findings and discusses the utility of imaging in the differential diagnosis of pediatric soft-tissue tumors.

This study presents visceral and skeletal imaging findings commonly observed in pediatric patients with type I Gaucher disease. Presented images show methods used for radiologic assessment of pediatric Gaucher patients, and imaging findings are discussed in the context of the underlying pathophysiology of the disease. Routine radiologic surveillance plays a central role in assessing Gaucher disease progression and response to treatment, but monitoring of pediatric patients presents specific challenges with regard to minimizing radiation exposure and interpreting extent of marrow involvement against the backdrop of normal growth-related changes in marrow composition. In addition to highlighting imaging findings in children with type I Gaucher disease, this manuscript discusses alternate modalities, which minimize radiation and may be just as accurate, if not better, than conventional methods exposing the child to radiation.