Fibromatosis, Congenital Generalized
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Synonyms of Fibromatosis, Congenital Generalized
- Infantile Myofibromatosis (IM)
- Congenital Multiple Fibromatosis
Congenital generalized fibromatosis (CGF) is a pediatric condition that is often now referred to as "infantile myofibromatosis" (IM). It is characterized by the formation of single or multiple noncancerous (benign) tumors that appear to be derived from cells forming certain supporting and binding tissues of the body and involuntary (smooth) muscle. These firm, nodular, potentially locally invasive tumors may involve the skin and underlying (subcutaneous) tissues, muscle tissue, bones, and/or certain internal organs (viscera).
In many cases, the tumors are present at birth (congenital), develop within the first few weeks of life, or may initially become apparent before the age of two years. Following initial growth and multiplication (proliferation) of tumor cells, the tumors usually eventually recede and disappear on their own (spontaneously). Those with solitary or multiple lesions without visceral involvement typically have a benign disease course. However, in infants with severe or widespread involvement of vital internal organs (i.e., multicentric, visceral involvement), potentially life-threatening complications may occur.
Fibromatosis, Congenital Generalized Resources
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