Involvement of the Bony Orbit in Infantile Myofibromatosis

Abstract

Infantile myofibromatosis is a rare disorder of infancy that can provoke osteolytic lesions. A 15-day-old infant presented with three round, firm lesions located on the forehead, shoulder, and back. Excisional biopsy of the forehead lesion revealed that the tumor was composed of spindle cells resembling normal smooth muscle arranged in short fascicles. Immunohistochemical staining was positive for vimentin and actin. Five months later, the child presented with three new lesions, including one in the superolateral aspect of the left orbit. It is important to recognize the multicentric form of infantile myofibromatosis because, despite its aggressive clinical presentation, the disease is benign and usually does not require extensive surgery or chemotherapy.

Solitary Intraosseous Orbital Myofibroma in Four Cases

“…The generalized form is often present at or shortly after birth as multiple nodules in the dermis, muscle, viscera, and bone. [3][4][5][6][7][8][9] Affected children have a guarded prognosis, and as many as 75% die with signs of respiratory distress or diarrhea soon after birth. 1 Many of the lesions can regress spontaneously.…”

Solitary Myofibroma of the Sclera

“…Myofibromas around the eye are rare and are generally limited to the eyelids and orbit. [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Our case is the first description of a solitary myofibroma involving the sclera, as far as we can determine through a search of the literature by using the PubMed search engine.…”

“…2,[7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] They all presented in the extraocular region, including eyelids, periorbital soft tissues, and orbital bones. To date, we are not aware of a report of infantile myofibromatosis involving the globe.…”

Solitary adult orbital myofibroma: Report of a case and review of the literature

“…The head and neck is the most common anatomic sub-region involved by myofibroma and the tumor is mainly seen in infancy and childhood. There are several case reports and series of infantile/childhood orbital myofibroma 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 and involvement of periocular soft tissues. 20 , 21 , 22 However, though myofibroma is the most common fibrous tumor of infancy, the tumor remains rare in the orbit.…”