title: Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma.
abstract: 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.
authors.affiliation: *Department of Ophthalmology, Weill Cornell Medical College; †Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, New York; ‡Implant and Prosthodontic Associates, Oklahoma City, Oklahoma; and §Department of Pediatrics, and ‖Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, New York, U.S.A.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Klufas
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Wolden
authors.forename: George C
authors.initials: GC
authors.lastname: Bohle
authors.forename: Leonard H
authors.initials: LH
authors.lastname: Wexler
authors.forename: David H
authors.initials: DH
authors.lastname: Abramson
mesh_terms: D001918:Brachytherapy / Q000295:instrumentation* / Q000379:methods; D017024:Chemotherapy, Adjuvant; D002648:Child; D006801:Humans; D007457:Iodine Radioisotopes / Q000627:therapeutic use; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy*; D017233:Orbit Evisceration; D020273:Orbital Implants; D009918:Orbital Neoplasms / Q000235:genetics / Q000473:pathology / Q000532:radiotherapy*; D061766:Proton Therapy; D034802:RNA-Binding Protein EWS / Q000235:genetics; D011879:Radiotherapy Dosage; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology / Q000532:radiotherapy*
chemical_list: D007457:Iodine Radioisotopes; D034802:RNA-Binding Protein EWS