Solitary Intraosseous Orbital Myofibroma in Four Cases
ABSTRACT 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.
- SourceAvailable from: Ann-Cathrine Larsen
Acta ophthalmologica 10/2010; 89(7):e600-1. DOI:10.1111/j.1755-3768.2010.02003.x · 2.84 Impact Factor
- "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. "
- Journal of AAPOS: the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus 06/2010; 14(3):203-4. DOI:10.1016/j.jaapos.2010.05.001 · 1.00 Impact Factor
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ABSTRACT: A 47-year-old male with history of schizophrenia developed painful proptosis and vision loss. Computed Tomography revealed a bone-destructive mass with encroachment on the orbit and compression of the right eye. Superolateral orbitotomy and biopsy revealed a lesion composed of a mixture of spindled and epithelioid cells without significant cytologic atypia or mitotic rate. Immunohistochemical stains were positive for vimentin and multifocally for smooth muscle actin, supporting the diagnosis of orbital myofibroma. Although orbital myofibromas typically present during childhood, they may occur in older patients and act as an expanding mass causing compression of adjacent structures.Orbit (Amsterdam, Netherlands) 02/2012; 31(1):21-3. DOI:10.3109/01676830.2011.605501