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infantile myofibromatosis |
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infantile myofibromatosis, a condition present at birth or occurring soon after, characterized by solitary or multiple firm, rubbery, spherical, or ovoid nodules in the skin and subcutaneous tissue. The nodules are composed of myofibroblasts and may undergo ulceration and calcification. In about half of patients, skeletal fibromas also occur. When lesions are limited to the skin and bones (a condition sometimes known as congenital multiple fibromatosis), prognosis is good and lesions resolve spontaneously. Visceral involvement may also occur (congenital generalized fibromatosis) and is often lethal. infantile myofibromatosis A condition characterized by fibrosis affecting soft tissue, bone, and viscera of infants and young children; IM is usually solitary but may be multicentric; it rarely recurs, and may regress with time. See Fibrous
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30 A subsequent review of the literature identified 170 patients with infantile myofibromatosis, of whom 42 had lesions present at birth. Microscopically, infantile fibromatosis must be differentiated from infantile myofibromatosis and infantile fibrosarcoma because their histologic resemblance is close. Infantile myofibromatosis is generally considered to be a benign process, unlike aggressive fibromatusis and fibrosarcomas, which are invasive lesions with metastatic potential. |
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