Myofibroma, the most common juvenile fibrous disorder in infancy, usually manifests as a solitary soft tissue
tumor and less commonly as simultaneous multiple
tumors in both soft tissue and bones. Infantile
myofibromatosis is well described in the head and neck, but cutaneous lesions rarely occur in the periorbit and orbit, where fast growth and bone damage can mimic malignant
tumors. We describe a case of a solitary periorbital
myofibroma in a newborn. Treatment consisted of partial excision of the
tumor. Histologic and immunohistochemistry analyses provided the diagnosis of infantile
myofibromatosis. The patient remains disease-free
at 10 years of age, but with some
visual impairment. Infantile
myofibromatosis is an uncommon
tumor with exceptional periorbital involvement. Differential diagnosis can be difficult when it is solely based on histologic assessment. Immunohistochemistry evaluation demonstrating cytoplasmic actin filaments in neoplastic spindle cells confirms the diagnosis. As soon as the diagnosis is made, chest and abdominal imaging must be performed to evaluate the overall prognosis and direct treatment. The treatment of choice is early conservative surgery to minimize functional and/or esthetic damage. Complete
tumor excision is not always possible. Lengthy ophthalmologic monitoring is required to detect the onset of
amblyopia.