MULTIDISCIPLINARY MANAGEMENT OF REFRACTORY ORBITAL RHABDOMYOSARCOMA

Purpose: Combined chemotherapy and radiation therapy have improved the survival of children with primary orbital rhabdomyosarcoma, but recur rence or persistence of the local orbital tumor still occurs. There ar e no established guidelines for dealing with these uncommon patients, and the authors present a review of the combined method treatment and outcome of children with refractory primary orbital rhabdomyosarcoma. Methods: From clinical databases, 67 children with orbital rhabdomyosa rcoma were identified. Seven (10%) of the 67 children had tumors refra ctory to combined chemotherapy and radiation therapy and underwent exe nteration or eye-sparing tumor excision. Their clinical course and out come were reviewed retrospectively. Results: No patient was lost to fo llow-up, which ranged from 3.2 to 11 years. Five (71%) of the seven ch ildren with refractory tumor are still alive at more than 3 years afte r surgery (3.2-11 years; mean, 6.9). In one of the two children who di ed, tumor extended beyond the operative margins at exenteration, and t he other child died with regional metastasis within a month of exenter ation.Conclusions: Although more than 90% of children with orbital rha bdomyosarcoma respond to combined therapy by pediatric oncologists and radiotherapists, local orbital (salvage) surgery by ophthalmologists may be of value in the minority of children with refractory tumors. Al l of the five surviving children appear to be disease free.