Role of imaging in atypical infantile lipofibromatosis

Abstract

Lipofibromatosis is an extremely rare, hamartomatous soft-tissue tumor seen almost exclusively in the pediatric age group. They are typically found in the extremities. Varied clinical presentations and lack of specificity in imaging findings often create a diagnostic dilemma. A systematic approach with emphasis on tumor composition is needed to arrive at a correct diagnosis. We report an unusual case of a giant lipofibromatosis that presented as a painless progressive back swelling in a 10-month-old male infant.