Orbital and periorbital myofibromas in childhood: two case reports.
OBJECTIVE
Infantile myofibromatosis is an uncommon tumor that occurs rarely in the periorbit and orbit. This article reports two cases of infantile myofibromatosis of the orbital adnexa and describes the associated clinical, histopathologic, and immunohistochemical findings.


METHODS
Two retrospective, interventional case reports with clinicopathologic correlation.


METHODS
Treatment consisted of excision of the tumors.


METHODS
Histologic and immunohistochemical evaluation and clinical evaluation for tumor recurrence.


RESULTS
The first patient was a newborn male with a large tumor extending from his eyelid that was excised at day 2 of life. Histologic and immunohistochemistry analyses were used to make a diagnosis of infantile myofibromatosis. He remains disease free at age 7 years. The second case was a 6-year-old boy with a 1-month history of proptosis resulting from an orbital mass. Incisional biopsy revealed a tumor consistent with infantile myofibromatosis. He remains tumor free 12 months after complete gross surgical resection.


CONCLUSIONS
Infantile myofibromatosis is an uncommon tumor that is rare in the orbit. Differential diagnosis can be difficult based solely on histologic analysis. Immunohistochemistry evaluation demonstrating cytoplasmic actin filaments within neoplastic spindle cells confirms the diagnosis. As soon as the diagnosis is made, chest and abdominal imaging is of value to define the prognosis and to direct further treatment. After the diagnosis of nonvisceral infantile myofibromatosis, complete gross resection, if possible, is the treatment of choice.
Westfall
Andrew C
AC
Department of Ophthalmology, Casey Eye Institute, Oregon Health and Sciences University, 3375 SW Terwilliger Boulevard, Portland, OR 97201, USA.
Mansoor
Atiya
A
Sullivan
Scot A
SA
Wilson
David J
DJ
Dailey
Roger A
RA
D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D018572:Disease-Free Survival; D006801:Humans; D007231:Infant, Newborn; D007889:Leiomyoma / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D009918:Orbital Neoplasms / Q000737:chemistry / Q000000981:diagnostic imaging / Q000473:pathology*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
D014408:Biomarkers, Tumor