Myofibromatose Infantile Cutanée Isolée

Solitary cutaneous infantile myofibromatosisInfantile myofibromatosis is the most frequent fibromatosis in childhood. It is a benign proliferation of fibroblasts and myofibroblasts. This case report concerns a newborn who presented at birth with a purple cutaneous nodule on the scalp. Surgical excision was performed at the age of 16 months. Infantile myofibromatosis was diagnosed on histology Infantile Myofibromatosis (IMF) ,as first described by Enzinger in 1981. Three types can exist. Solitary MFI, the most frequent, is a solitary lesion, cutaneous/subcutaneous, osseous or involving soft tissues. Multicentric disease is caracterized by multiple locations and generalized form by visceral involvement. Morphological features are identical in aft The histological diagnosis relies on the identification of two separate components, a fascicular myofibroblastic pattern at the periphery with a hemangiopericytoma like pattern in the centre. Both are positive for alpha-smooth muscle actin. Atypia, or mitotic activity, are not observed usually but features of intravascular growth can be seen in the centre of the lesion.Infantile MF carries a good prognosis when solitary but death frequently occurs in generalized MF with visceral involvement.