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Orbital rhabdomyosarcomas: A review
Authors:Lama Jurdy  Johanus H.M. Merks  Bradly R. Pieters  Maarten P. Mourits  Roel J.H.M. Kloos  Simone D. Strackee  Peerooz Saeed
Affiliation:1. Orbital Centre, Department of Ophthalmology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands;2. Department of Paediatric Oncology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands;3. Department of Radiotherapy, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands;4. Department of Plastic and Reconstructive Surgery, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Abstract:
Rhabdomyosarcoma (RMS) is a highly malignant tumor and is one of the few life-threatening diseases that present first to the ophthalmologist. It is the most common soft-tissue sarcoma of the head and neck in childhood with 10% of all cases occurring in the orbit. RMS has been reported from birth to the seventh decade, with the majority of cases presenting in early childhood. Survival has changed drastically over the years, from 30% in the 1960’s to 90% presently, with the advent of new diagnostic and therapeutic modalities. The purpose of this review is to provide a general overview of primary orbital RMS derived from a literature search of material published over the last 10 years, as well as to present two representative cases of patients that have been managed at our institute.
Keywords:Rhabdomyosarcoma  Tumor  Orbit  Eye  AMORE  Genetics  Pediatrics  Brachytherapy
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