CONGENITAL SOLITARY FIBROMATOSIS OF SOFT TISSUES, A VARIANT OF CONGENITAL GENERALIZED FIBROMATOSIS

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Abstract
A report of 2 cases of solitary fibromatosis in a 10 day old boy and a girl 3 yr, 10 mo. old is presented. Both lesions were deep-seated and showed a nodular and infiltrating growth, predominantly built-up by immature fibroblast-like cells and including hemangiopericytoma-like areas. One of the lesions also showed leiomyoma-like areas. An ultrastructural study revealed no intra-cytoplasmatic myofilaments. At follow-up examinations after 21 yr and 1 yr, respectively, there were no signs of recurrences or metastases. These 2 cases are considered to represent a solitary form of congenital generalized fibromatosis. The differential diagnosis from infantile hemangiopericytoma and fibrous lesions seen in infancy and early childhood, such as infantile fibrosarcoma, diffuse infantile fibromatosis, extra-abdominal desmoid, fibrous hamartoma of infancy and juvenile aponeurotic fibroma, is discussed.