Myxofibrosarcoma of the Orbit: An Underrecognized Entity? Case Report and Review of the Literature
Received 20 June 2007; received in revised form 27 September 2007; accepted 19 October 2007. published online 21 December 2007.
Purpose
To describe the clinicopathologic and immunohistochemical features of a rare case of myxofibrosarcoma (MFS) involving the orbit.
Design
Retrospective interventional case report and review of literature.
Methods
The clinical history, systemic imaging studies, and histopathologic results of the orbital biopsy are reviewed.
Main Outcome Measures
Clinical, radiologic, and histologic features of MFS.
Results
A 63-year-old Chinese male sought treatment for a history of rapidly progressive proptosis associated with decreased vision in the left eye over 10 days. Magnetic resonance imaging of the orbit revealed a homogenous mass extending to the optic canal. The mass was excised partially and was submitted for histologic and ultrastructural examination. Histologic testing revealed features of MFS with characteristic diffuse proliferation of atypical, spindle-shaped fibroblasts with prominent myxoid stroma. Tumor cells were reactive for vimentin. Ultrastructural examination revealed spindle-shaped tumor cells, rich in dilated rough endoplasmic reticulum and prominent vacuoles.
Conclusions
For historical reasons, MFS involving the orbit is likely to be underrecognized and categorized erroneously as malignant fibrous histiocytoma or other soft tissue sarcoma. Although orbital MFS is an extremely rare diagnosis, ophthalmologists should be aware of the possibility and consider a diagnosis of MFS when an adult seeks treatment for an orbital tumor with aggressive growth.
Available online: December 21, 2007.
1Doheny Eye Institute, Keck School of Medicine of the University of Southern California, Los Angeles, California.
2Armed Forces Institute of Pathology, Washington, DC.
Correspondence to Narsing A. Rao, MD, 1450 San Pablo Street, DVRC 211, Los Angeles, CA 90033.
Manuscript no. 2007-825.
Supported by Research to Prevent Blindness, Inc., New York, New York (NAR). Dr Khurana is a Heed Fellow and is supported by Heed Ophthalmic Foundation, Cleveland, Ohio.
No author has a proprietary interest in the article's subject.
ABSTRACT