The name infantile myofibromatosis is a misnomer because the most common form of this disorder is solitary, not multicentric as the name implies.
Infantile myofibromatosis is a benign neoplasm of infancy, usually present at birth. Clinically it presents as a solitary nodule 70-80 percent of the time, most commonly on the head, neck and trunk. The multicentric forms may have visceral involvement with an increase in mortality. Histology shows an unencapsulated, well-circumscribed lobule of peripheral spindle cells, resembling smooth muscle. A central area with blood vessels surrounded by small rounded cells is also often seen, giving infantile myofibromatosis its biphasic appearance. Treatment has traditionally consisted of radical excision, but recent evidence shows that solitary lesions may regress. Therefore, a watch and wait approach may be warranted in certain cases .
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