Lipofibromatosis : Introduction

Introduction: Lipofibromatosis (infantile fibromatosis) is an uncommon fibroblastic lesion of infancy and early childhood. It accounts for 5% to 10% of fibrous tumors of childhood and usually involves hands and feet.

It consists of mature adipose tissue, infiltrating bundles of bland fibroblastic cells, and lipoblast-like cells at the interface between lipomatous and spindle cell areas. Recent molecular studies have suggested involvement of PI3K/AKT/mTOR pathway in its pathogenesis.

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