title: [Orbital Exenteration in Patient with Metastatic Choroidal Melanoma - a Case Report].
abstract: BACKGROUND
  Uveal melanoma is the most common primary intraocular tumour in adults in Caucasians and in 75% is arising from choroid. It threatens not only the patients loss of vision and eye, but also 50% of patients after 5-year interval after therapy die due to distant metastases. The treatment of small and medium-sized melanoma are methods preserving eye globe. Almost half of the total number of patients is still unavoidable enucleation. Considerably rarer is indicated exenteration of an orbit. These tumors metastasize only hematogenous, while the most frequent place of localization of distant metastases is the liver. Generalized disease prognosis is poor, and our current treatment options in this stage are ineffective.
  
  
  METHODS
  Case report of 59 years old patient with choroidal melanoma stage T4 N1 M1 massively infiltrating the orbit. At the time of diagnosis of the primary tumor distant metastases were present. The patient underwent exenteration of the orbit and systemic chemotherapy.
  
  
  CONCLUSIONS
  Although choroidal melanomas with extrascleral extension and infiltration into the orbit have no better prognosis after exenteration of the orbit, surgery is providing us local tumour control. Good cosmetic effect after this mutilating procedure is offered by individually made prosthesis (epithesis). All patients with uveal melanoma require lifelong dispensation, distant metastases may occur even after many years. In the treatment of generalized disease is available systemic chemotherapy and immunotherapy only palliative. The best effect on survival has complete surgical resection of single metastasis. Uveal melanoma has a different genetic profile as cutaneous melanoma. The biological nature of uveal melanoma seems to be the key to determining risk patients, as well as the development of targeted systemic therapy.
  
  
  CONCLUSIONS
  Treatment of patients with generalized large uveal melanoma with extrascleral extension is difficult. A better understanding of biological interest may be the key to the detection of patients at higher risk of distant metastases formation, and to an effective systemic treatment.
  
  
  BACKGROUND
  large uveal melanoma, extrascleral extension, orbital exenteration, the treatment of generalized disease.
authors.forename: P
authors.initials: P
authors.lastname: Justusová
authors.forename: M
authors.initials: M
authors.lastname: Štubňa
authors.forename: M
authors.initials: M
authors.lastname: Veselovský
authors.forename: B
authors.initials: B
authors.lastname: Lipková
mesh_terms: D002830:Choroid Neoplasms / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008545:Melanoma / Q000556:secondary / Q000601:surgery*; D008875:Middle Aged; D009915:Orbit / Q000601:surgery; D017233:Orbit Evisceration; D009918:Orbital Neoplasms / Q000556:secondary / Q000601:surgery*; D019736:Prostheses and Implants; D016896:Treatment Outcome