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CONGENITAL MULTIPLE FIBROMATOSIS

A CASE REPORT WITH REVIEW OF THE WORLD LITERATURE

JEANNE W. BAER M.D. and MARY ANN RADKOWSKI M.D.

A case of congenital multiple fibromatosis is reportd.

Although rare, it should be a major consideration in the differential diagnosis of multiple well circumscribed lytic lesions affecting the diaphysis and metaphysis of long bones as well as the skull in the newborn period.

A review of the literature points out 2 groups of patients with the same locally infiltrating fibromas. They become differentiated by the degree of visceral involvement which determines survival. Those patients with congenital generalized fibromatosis will die within the first 4 months of life 80 per cent of the time. Patients without visceral involvement do not die. Therefore, they have been separated into a group called congenital multiple fibromatosis. The clinical course can be well correlated with the status of the bone lesions as seen on the roentgenograms.


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