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We appreciate the comments from Dr Fay and colleagues regarding our article.¹ Their assertion that exenteration is not a primary treatment modality for children with orbital rhabdomyosarcoma is correct. However, this surgical approach remains an important treatment alternative in patients in whom initial therapy fails, as it did in this child.

The patient described in case 2 was referred to the pediatric oncology service at diagnosis and the case was presented on several subsequent occasions to the pediatric tumor board at the University of California, San Francisco. He was initially treated with 2 cycles of vincristine sulfate and dactinomycin for embryonal rhabdomyosarcoma (stage I, group 3) confined to the left orbit. Despite this therapy, he had clinical and radiographic evidence of disease progression. His therapy was changed to ifosfamide and etoposide, with orbital radiation therapy. Follow-up evaluations showed continued progression. A surgical exenteration was performed for failure to . . . [Full Text of this Article]

Joan M. O'Brien, MD; Seymour Zoger, MD; Katherine K. Matthay, MD; Robert M. Goldsby, MD
San Francisco, Calif

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