Myxofibrosarcoma of the orbit: an underrecognized entity? Case report and review of the literature.
OBJECTIVE
To describe the clinicopathologic and immunohistochemical features of a rare case of myxofibrosarcoma (MFS) involving the orbit.


METHODS
Retrospective interventional case report and review of literature.


METHODS
The clinical history, systemic imaging studies, and histopathologic results of the orbital biopsy are reviewed.


METHODS
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.
Wang
Mingwu
M
Doheny Eye Institute, Keck School of Medicine of the University of Southern California, Los Angeles, California, USA.
Khurana
Rahul N
RN
Parikh
Jignesh G
JG
Hidayat
Ahmed A
AA
Rao
Narsing A
NA
D014408:Biomarkers, Tumor / Q000032:analysis; D003937:Diagnosis, Differential; D005094:Exophthalmos / Q000175:diagnosis; D017809:Fatal Outcome; D005354:Fibrosarcoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D013902:Radiography, Thoracic; D012189:Retrospective Studies
D014408:Biomarkers, Tumor