Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma Academic Article uri icon


MeSH Major

  • Brachytherapy
  • Neoplasm Recurrence, Local
  • Orbit Evisceration
  • Orbital Implants
  • Orbital Neoplasms
  • Sarcoma, Ewing


  • A 6-year-old boy initially presented to an outside hospital with a right orbital mass with biopsy positive for translocation involving EWS RNA-binding protein 1 gene and imaging consistent with primary extraskeletal Ewing sarcoma (ES). There was no evidence of metastatic disease. Patient underwent gross tumor resection and adjuvant chemotherapy (VAdriaC/IE) followed by postoperative 45-Gy proton beam radiation. After 19 months, a solitary in-field local recurrence occurred, which was unsuccessfully surgically resected. Thereafter, treatment commenced with irinotecan and temozolomide, and the patient presented to the center of the authors. MRI showed locally recurrent disease without evidence of metastatic disease. Right orbital exenteration was performed, and an orbital mold was fashioned to deliver brachytherapy. There were no complications. The patient had no evidence of recurrent disease at 37-month follow up. This is the first report of orbital implant brachytherapy for recurrent primary ES of the orbit, and an additional report of primary extraskeletal ES of the orbit, which is a rare primary orbital tumor.

publication date

  • January 2015



  • Academic Article



  • eng

PubMed Central ID

  • PMC5014374

Digital Object Identifier (DOI)

  • 10.1097/IOP.0000000000000111

PubMed ID

  • 24814278

Additional Document Info

start page

  • e89

end page

  • 91


  • 31


  • 4