Solitary Intraosseous Orbital Myofibroma in Four Cases
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. Ophthalmic Plastic and Reconstructive Surgery
(Impact Factor: 0.88).
07/2006; 22(4):292-5. DOI: 10.1097/01.iop.0000226900.79557.cf
To report four pediatric cases of a bone-destructive orbital tumor that proved to be a benign solitary intraosseous myofibroma.
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.
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.
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.
Available from: Ann-Cathrine Larsen
- "Solitary myofibroma is usually a nontender and painless tumour, with or without orbital bone involvement, and can be rapidly progressing or slow growing (Chung & Enzinger 1981; Persaud et al. 2006; Rodrigues et al. 2006). The clinical and histopathological differential diagnoses of infantile myofibroma include solitary fibrous tumour, nodular fasciitis, fibrous histiocytoma , haemangiopericytoma, leiomyoma and leiomyosarcoma (Bernardini et al. 2003; Rodrigues et al. 2006). These conditions share a number of characteristics, but some immunohistological difference helps in the differential diagnosis. "
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ABSTRACT: This describes a non-interventional case series of 2 patients, aged 7 and 9 years referred to Oculoplastic Unit, both for evaluation of a gradually enlarging, painless, mass of the cheek. CT scan of the first case revealed left orbital floor destruction from a well-defined intraosseous mass. The second was a round circumscribed orbital floor tumor without bone destruction. Histological diagnosis of myofibroma was rendered in both cases. Solitary myofibromas are rare in the orbit. Their rapid growth and bony destruction can mimic malignant tumors. Complete excision with close follow-up is the preferred treatment. Solitary myofibroma should be considered in the differential diagnoses of fibrous tumors with bone destruction in the orbit.
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