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Primary orbital melanoma: a case series and literature review
Authors:Edwin Figueira  Saul Rajak  Penny McKelvie  George Kalantzis  Azzam Ismail  Michael Gonzales
Affiliation:1. South Australian Institute of Ophthalmology, Royal Adelaide Hospital, Adelaide, Australia;2. Department of Ophthalmology, Prince of Wales Hospital, Randwick, NSW, Australiafigueirae@gmail.com;4. The Sussex Eye Hospital, Brighton and Sussex University Hospital, Brighton, UK;5. Department of Anatomical Pathology, St Vincent’s Hospital, Victoria, Australia;6. Department of Ophthalmology, Leeds Teaching Hospital, Leeds, UK;7. Histopathology Department, Leeds Teaching Hospital NHS Trust, Leeds, UK;8. Department of Anatomical Pathology, Royal Melbourne Hospital, Melbourne, Victoria, Australia
Abstract:Primary orbital melanoma (POM) is a very rare condition. We report further four cases and review all previously reported cases. We present a multicentre retrospective review of patients with POM. Clinical, radiological, surgical, histological, and follow-up data is presented. Four patients with POM were identified between 2000 and 2013. All presented with proptosis and diplopia without reduced vision. Two had known pre-existing blue cell naevi. All were stage T1N0M0. All underwent exenteration with adjuvant radiotherapy. All are disease free at follow-up durations of 24–151 months. The present three cases and review of all cases in the literature suggest a higher likelihood of disease-free survival from primary exenteration (7/8 disease-free survival, 1/8 death from metastatic disease) than wide local excision (7/16 disease-free survival, 9 recurrence or metastasis of whom 4 died). Adjuvant radiotherapy may additionally improve outcomes.
Keywords:Adjuvant treatment  blue cellular naevus  congenital ocular melanocytosis  exenteration  orbit  primary orbital melanoma
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