title: Extraskeletal osteosarcoma of the orbit: A clinicopathologic case report and review of literature.
abstract: Primary extraskeletal osteosarcoma (EOS) is an extremely rare malignancy. In this report, the clinical course of a 32-year-old man presenting with proptoses is described. Medical history included Hirschsprung disease (HD), horseshoe kidney, azoospermia, and vertebral anomalies. Imaging of the orbit showed an oval, well-defined heterogeneous mass adjacent to the lateral wall of the orbit. The patient underwent a lateral orbitotomy and complete excision of the mass. The mass was not attached to the bone. Histopathologic and immunohistochemical examination confirmed the diagnosis of an EOS. The patient received chemotherapy and radiotherapy and is free of the disease 3 years after the diagnosis. Genetic screening showed no mutations for both the RET proto-oncogene for HD and the p53 tumor suppressor gene for osteosarcoma.
authors.affiliation: Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium.
authors.forename: V M D S
authors.initials: VM
authors.lastname: de Maeyer
authors.affiliation: Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium.
authors.forename: P A F A
authors.initials: PA
authors.lastname: Kestelyn
authors.affiliation: Department of Orbit and Oculoplasty, Bombay City Eye Hospital, Mumbai, India.
authors.forename: Akash D
authors.initials: AD
authors.lastname: Shah
authors.affiliation: Department of Pathology, Ghent University Hospital, Ghent, Belgium.
authors.forename: C M
authors.initials: CM
authors.lastname: Van Den Broecke
authors.affiliation: Department of Medical Oncology, Ghent University Hospital, Ghent, Belgium.
authors.forename: H G N
authors.initials: HG
authors.lastname: Denys
authors.affiliation: Department of Ophthalmology, Ghent University Hospital, Ghent, Belgium.
authors.forename: C E
authors.initials: CE
authors.lastname: Decock
mesh_terms: D000328:Adult; D005094:Exophthalmos / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013508:Ophthalmologic Surgical Procedures; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D012516:Osteosarcoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D000090063:Proto-Oncogene Mas; D014057:Tomography, X-Ray Computed; D014792:Visual Acuity / Q000502:physiology