[Role of conservative surgery in the multidisciplinary treatment of Ewing's sarcoma in childhood].

BACKGROUND

In order to cure Ewing's sarcoma it is necessary to have an approach which considers the radical local control on the sites of macroscopic disease, along with the systemic control of micrometastases. On the present study the experience of the authors in analyzed, remarking the role of cytoreduction surgery on curability.

METHODS

From January 1982 to August 1991, 24 patients with the mean age 13 years, 14 boys and 10 girls, previously untreated and with a pathology proven diagnosis have been treated by the authors. The treatment protocol included: alternating chemotherapy with cyclophosphamide, adriamycin, methotrexate, bleomycin, actinomycin D and vincristine; administered simultaneously with preoperative external radiation with a volume that completely included the affected bone and surrounding soft tissues for a total dose of 45 Gy/4.5 weeks. After a resting period of 4 weeks, resection of the involved bone and adjacent healthy bone was performed, followed by a single dose of 10-15 Gy of intraoperative radiotherapy to the tumor bed. Subsequently a custom prostheses or allograft was implanted.

RESULTS

Twenty patients had localized disease and 4 had metastatic disease at diagnosis. In 16 cases the tumor was in extremities, 5 axial, and 3 extraskeletical. In 15 patients surgery with limb sparing techniques was performed, 8 had en block resection and one amputation (calcaneous location). At the time of this report 21 patient are alive (87%). Four had disease progression, of this 3 had died (12%). The actuarial disease free survival rate is 80% +/- 9% with a follow-up of 104 months, being the mean survival time of 85.5 months.

CONCLUSIONS

The cytoreduction surgery included into a multidisciplinary approach permits to achieve a high rate of cure in Ewing's sarcoma. The toxicity of the program can be considered acceptable.