Solitary intraosseous orbital myofibroma in four cases.
OBJECTIVE
To report four pediatric cases of a bone-destructive orbital tumor that proved to be a benign solitary intraosseous myofibroma.


METHODS
Four children, aged 1, 7, 11, and 72 months, developed eyelid fullness or slight proptosis. A solitary intraosseous mass was detected in the inferior (n = 2), superotemporal (n = 1), and supranasal (n = 1) orbital walls. On CT, the mass showed bone destruction. On MRI, the mass appeared circumscribed within a shell of normal surrounding bone and with features of isointensity on T1-weighted images, intense homogeneous gadolinium enhancement, and midrange signal on T2-weighted images. Complete surgical excision of the mass in each case was performed.


RESULTS
On histopathologic examination, the tumors were characterized by whorls and bands of plump myoid cells with eosinophilic cytoplasm consistent with myofibroblasts, sometimes associated with a hemangiopericytoma-like vascular pattern. The diagnosis was also supported by immunohistochemical studies showing positive immunoreactivity for vimentin and smooth muscle actin. None of the patients have shown recurrence in follow-up ranging from 6 months to 3 years.


CONCLUSIONS
Myofibroma is a benign tumor that can occur in the orbital bony wall of children. It can manifest as a bone destructive mass and can simulate a malignant process.
Rodrigues
Eduardo B
EB
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.
Shields
Carol L
CL
Eagle
Ralph C
RC
Marr
Brian P
BP
Shields
Jerry A
JA
D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D047708:Myofibroma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed