infantile myofibromatosis

in·fan·ti·le my·o·fi·bro·ma·to·sis

myofibromatosis seen at birth or in infants, with multiple lytic bone lesions and involving soft tissue, or with visceral involvement.

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 tumor of childhood.
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5,6) Infantile myofibromatosis typically involves the mandible and consists of a circumscribed nodule with short fascicles of actin-positive cells often admixed with hemangiopericytoma-like vessels.
INTRODUCTION: Infantile myofibromatosis is a rare mesenchymal neoplasm of infancy and early childhood, typically presenting as single or multicentric nodular masses of soft tissues, bones, or visceral organs.
Features were suggestive of infantile myofibromatosis solitary form.
DISCUSSION: Infantile myofibromatosis is a rare mesenchymal tumour usually found in the first decade of life with 88% of cases detected before the age of 2 years and 60% at or shortly after birth.
Study of 199 cases of infantile myofibromatosis by Jenkins and Cawley (11) revealed that the multicentric form (56%) was more common than the solitary form (44%).
Although there has been no documentation describing the mechanism of the spontaneous regression of infantile myofibromatosis, Fukasawa et al.
Immunostaining for smooth muscle actin and vimentin, with negative staining for desmin can support the myofibroblastic differentiation of infantile myofibromatosis.
9) They show a considerable morphologic overlap with related tumors such as infantile myofibromatosis, solitary adult myofibroma, glomangiopericytoma, glomangiomyoma, and some vascular leiomyomas (angioleiomyomas).
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.
In 1981, Chung and Enzinger published a review of 61 cases of infantile myofibromatosis and described only 2 patients with a solitary myofibroma of the cheek or parotid area.

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