title: Malignant peripheral nerve sheath tumor of the orbit: malignant transformation from neurofibroma without neurofibromatosis.
abstract: A 52-year-old woman suffered from progressive proptosis and vision loss due to a large tumor in her right orbit. Multiple recurrences of the tumor were treated with surgical excision. The pathologic diagnosis in each recurrence was neurofibroma, and the tumor transformed to malignant peripheral nerve sheath tumor in the final pathologic diagnosis. Orbital exenteration and postoperative irradiation were applied and there has been no evidence of tumor recurrence 5 years postoperatively.
authors.affiliation: Department of Ophthalmology, Taiwan Adventist Hospital, Taiwan.
authors.forename: Shuo-Fang
authors.initials: SF
authors.lastname: Cheng
authors.forename: Yun-I
authors.initials: YI
authors.lastname: Chen
authors.forename: Cheng-Yu
authors.initials: CY
authors.lastname: Chang
authors.forename: Yeh
authors.initials: Y
authors.lastname: Peng
authors.forename: Shu-Lang
authors.initials: SL
authors.lastname: Liao
mesh_terms: D001766:Blindness / Q000209:etiology; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D009455:Neurofibroma / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery; D017253:Neurofibromatoses / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D009918:Orbital Neoplasms / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery