title: Myxofibrosarcoma of the orbit: an underrecognized entity? Case report and review of the literature.
abstract: 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.
authors: Doheny Eye Institute, Keck School of Medicine of the University of Southern California, Los Angeles, California, USA.
authors: Mingwu
authors: M
authors: Wang
authors: Rahul N
authors: RN
authors: Khurana
authors: Jignesh G
authors: JG
authors: Parikh
authors: Ahmed A
authors: AA
authors: Hidayat
authors: Narsing A
authors: NA
authors: Rao
mesh_terms: 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
chemical_list: D014408:Biomarkers, Tumor