Multidisciplinary treatment of primary orbital rhabdomyosarcoma: a single-institution experience

Overall, rhabdomyosarcomas are not common, but they are among the most frequent tumors of muscle tissue. Among children, rhabdomyosarcomas account for four to eight percent of cancers. While many rhabdomyosarcomas have a poor prognosis, patients enjoy a far better prognosis if the disease is limited to the eye socket, or orbit. Published studies indicate a two-year survival rate of about 90 percent. In a retrospective evaluation of the treatment of 12 children, the authors indicate that the current treatment method, which combines radiotherapy with chemotherapy, can be completely effective when properly applied. Ten of the 12 patients were immediately treated with radiation and combined chemotherapy upon diagnosis, and two patients were treated with radiotherapy upon recurrence. The authors view the treatment of these two patients as corrective, in that the initial treatment was not properly applied. All 12 patients are alive without evidence of disease at follow-up periods ranging from 7 to 123 months. Eleven of the 12 patients received a radiation dose of 6,000 cGy (a Gy, or Gary, is one Joule of energy absorbed per kilogram of tissue). This level of radiation, when administered to the eye, is believed to cause cataracts. In the present series of patients, cataract formation was observed in only one patient, and lens opacities occurred in another two. An additional eight patients retain good visual acuity; the remaining patient had almost total lack of vision prior to treatment. The excellent preservation of vision is attributed to immediate treatment with radiotherapy. The ability of the eye to recover from radiation exposure may be compromised if radiotherapy is delayed until after chemotherapy. Therefore, treatment should not be delayed once a diagnosis of rhabdomyosarcoma of the orbit is confirmed. The authors suggest that the currently available treatment methods are already optimal and require only proper administration. (Consumer Summary produced by Reliance Medical Information, Inc.)