Exenteration and custom implant brachytherapy as a treatment for recurrent primary extraskeletal orbital Ewing sarcoma Journal Article


Authors: Klufas, M. A.; Wolden, S. L.; Bohle, G. C. 3rd; Wexler, L. H.; Abramson, D. H.
Article 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. Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Keywords: child; human tissue; preschool child; cancer surgery; cancer recurrence; case report; postoperative period; doxorubicin; cancer combination chemotherapy; cancer adjuvant therapy; cancer patient; radiation dose; temozolomide; nuclear magnetic resonance imaging; follow up; cytoreductive surgery; etoposide; cyclophosphamide; vincristine; ifosfamide; irinotecan; ewing sarcoma; rna binding protein; brachytherapy; proton radiation; evisceration; orbit implant; brachytherapy implant; human; male; priority journal; article; primary extraskeletal orbital ewing sarcoma
Journal Title: Ophthalmic Plastic and Reconstructive Surgery
Volume: 31
Issue: 4
ISSN: 0740-9303
Publisher: Lippincott Williams & Wilkins  
Date Published: 2015-07-01
Start Page: e89
End Page: e91
Language: English
DOI: 10.1097/iop.0000000000000111
PROVIDER: scopus
PUBMED: 24814278
PMCID: PMC5014374
DOI/URL:
Notes: Export Date: 2 September 2015 -- Source: Scopus
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MSK Authors
  1. Suzanne L Wolden
    560 Wolden
  2. Leonard H Wexler
    191 Wexler
  3. David H Abramson
    389 Abramson