RESUMO
UNLABELLED: Ewing's sarcoma and PNET are more and more frequently submitted to surgical treatment, as this method of local therapy seems to offer a better chance for cure. Which method surgery or radiotherapy (RTx) is more mutilating in childhood, should be carefully considered. The aim of the report is to review the possibility of local resection and reconstruction in various sites of the tumour. PATIENTS: 12 patients with Ewing's sarcoma / PNET were submitted to the site-specific surgical procedures and assessed for local control and function. All were pre-treated with CHT and continued that treatment after surgery. Four patients had fibular Ewing's sarcoma and all underwent subtotal fibulectomy. Long term complete remission (CR) was achieved in all. Two patients had Askin tumours; both developed pleural relapses distant to the primary tumour site. One patient had total scapulectomy (CR for 7 years). One patient had hemipelvectomy supplemented with RTx because of micro residue (CR for 10 years). Two patients were amputated: one for PNET of tibia invading nerves and vessels, another one for Ewing's sarcoma of femur. The first one developed pulmonary metastases and died one year later, the second one has been alive for 13 years but has badly accepted the prosthesis. Two patients with very extensive tumours of pelvis could never be operated on, they never achieved CR despite chemo- and radiotherapy and died. SUMMARY: Of 10 patients submitted to surgery 7 patients are alive and 4 have an excellent functional result. Of the 2 patients in whom the tumour was inoperable neither is alive. It seems justified to recommend surgical resection of Ewing's sarcoma / PNET; satisfying reconstruction is usually feasible. RTx seems to have value rather as a method supplementing minor failures of surgery than as the only local treatment.
