title: Primary pleomorphic liposarcoma of the orbit: a case report.
abstract: To our knowledge, pleomorphic liposarcoma (PL) of the orbit has only been reported in the literature four times. This rarity makes it more difficult to diagnose and to treat in this clinical setting. A 62-year-old female presented with pruritus, edema, proptosis and diplopia 5 months OS. Imaging revealed an intraorbital mass displacing the globe, with infiltration into the sinus. The tumor was removed and the histological examination revealed a highly cellular tumor with heterogenous histology, with a few vacuolated cells and many malignant features. Immunohistochemistry allowed for the differential diagnosis, resulting in a diagnosis of PL of the orbit. The cells were immuno-positive for S-100 and negative for all other relevant markers. According to the literature, prognosis for this neoplasm is quite poor, and exenteration represents the best treatment option. The patient refused exenteration and radiation therapy, however, at 2 year follow-up, she remained recurrence-free.
authors.affiliation: Henry C. Witelson Ocular Pathology Laboratory, McGill University, Montreal, Quebec, Canada.
authors.forename: Madeline
authors.initials: M
authors.lastname: Doyle
authors.forename: Alexandre N
authors.initials: AN
authors.lastname: Odashiro
authors.forename: Patrícia R
authors.initials: PR
authors.lastname: Pereira
authors.forename: Macanori
authors.initials: M
authors.lastname: Odashiro
authors.forename: Fernando
authors.initials: F
authors.lastname: Kobayashi
authors.forename: Ilton
authors.initials: I
authors.lastname: Shinzato
authors.forename: Miguel N
authors.initials: MN
authors.lastname: Burnier
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008080:Liposarcoma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D009418:S100 Proteins / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D009418:S100 Proteins