Group of disorders characterized by expanding benign fibrous tissue that are more aggressive than benign isolated fibromas and less aggressive than fibrosarcoma. Other names include “musculoaponeurotic fibromatosis,” referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal muscle, aggressive fibromatosis and “desmoid tumor.” A clear difference should be made between intraabdominal and extraabdominal localizations. Fibromatosis is a different entity from neurofibromatosis.
Fibromatosis Syndrome: Congenital fibromatosis affecting the scalp in an infant.
Fibromatoses can be divided in three syndromes:
Hereditary Desmoid Disease (Gardner Syndrome): Characterized by the presence of benign intraabdominal desmoid tumors that can be locally aggressive as a result of vascular or major organ compression which can lead to significant morbidity. Desmoid tumors can also arise sporadically.
Congenital Generalized Fibromatosis: Characterized by the presence of fibrous nodules that involve the skin, bone, and viscera. This medical condition is associated with significant mortality rate established as high as 80% when visceral nodules cause external compression/obstruction of surrounding vital organs. If the fibromas involve only the skin and skeleton and not the viscera, the disease is known as “Congenital Multiple Fibromatosis,” and the prognosis is excellent. In both conditions, it is believed that there are variants of the same disease process. The fibromas resolve completely and spontaneously.
Jaffe Campanacci Syndrome: Characterized by the presence of complex multiple nonossifying fibromas of the long bones, mandibular giant cell lesions, and café-au-lait macules without the usual neurofibromas.