title: [Epithelioid sarcoma of the orbit].
abstract: BACKGROUND
  Sarcomas count among the very rare malignancies of the orbit. Epithelioid sarcomas typically occur in the subcutaneous tissue of younger patients; an affected orbit constitutes a rarity.
  
  
  OBJECTIVE
  Only three cases of this extremely rare disease of the orbit, which is associated with marked aggressiveness and a very poor prognosis, have been analysed in the worldwide current literature.
  
  
  METHODS
  We present a case report together with a literature review. A rapidly progressing painful proptosis with visual loss occurred in a 30-year-old patient. Imaging and invasive diagnostic procedures were initiated as a consequence of this.
  
  
  RESULTS
  Diagnostic imaging revealed an infiltrative process of the right orbit. Following endonasal decompression of the orbit and probe acquisition, histological and immunohistochemical tests showed the presence of a proximal type epithelioid sarcoma. Therapy consisted of orbit exenteration together with implantation of titanium miniplates for orbital prosthesis. During the course of disease, osseous metastases developed. The patient died during palliative chemotherapy, 14 months after the initial diagnosis.
  
  
  CONCLUSIONS
  Epithelioid sarcomas are extremely rare, difficult to treat tumours in the head and neck region. The associated mortality rates are high. Aggressive surgical therapy with intensive follow-up is recommended. The prognosis depends upon the resection status.
authors: Klinik für Hals-, Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie, Plastische Operationen, Städtisches Klinikum Dessau, Auenweg 38, 06847, Dessau-Roßlau, Deutschland.
authors: M
authors: M
authors: Thranitz
authors: T
authors: T
authors: Berg
authors: C
authors: C
authors: Kneifel
authors: K
authors: K
authors: Stock
authors: S
authors: S
authors: Knipping
mesh_terms: D000328:Adult; D001766:Blindness / Q000175:diagnosis / Q000209:etiology*; D003937:Diagnosis, Differential; D005094:Exophthalmos / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009918:Orbital Neoplasms / Q000150:complications / Q000175:diagnosis*; D012509:Sarcoma / Q000150:complications / Q000175:diagnosis*