Publication de Nicole Delepine : Ewing's sarcoma (EW): long term favourable outcome in children with combined modality therapy and conservative surgery

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Publié sur : Journal of Clinical Oncology, ASCO Annual Meeting Proceedings. Vol 23, No. 16S, Part I of II (June 1 Supplement - Abstract N° 8561) - 2005
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Ewing's sarcoma (EW): long term favourable outcome in children with combined modality therapy and conservative surgery
F.Delépine, H.Cornille, S.Alkallaf, A.Tabbi, G.Delépine, Nicole Delepine

Ewing's sarcoma (EW) : long term favourable outcome in children with combined modality therapy and conservative surgery


Pediatric Oncology / Pediatric Solid Tumors


Abstract :


Background:
To assess the effect of surgery on local tumor control of EW in children, with localized disease. 25 years ago, many authors emphasized the crucial role of surgery in EW. Recently, others plae again for radiotherapy in local control.

Methods:
21 patients (p.) <18 y received DD2 protocol already described: short induction -6weeks-, systematic surgery and six alternate drugs (cyclophosphamide, pirarubicine, ifosfamide, cisplatine, vincristine-actinomycine D). We calculated the cumulated incidence of disease recurrence, late complications, overall and event free survival.

Results:
out of the 39 p. treated on first hand from Nov 1985 to Jan 2000, 21 were under 18 years at diagnosis, 13 girls, 8 boys. The median tumor volume was 284 cc. Locations were rib (4), femur (4), maxillar (1), radial (1), tibial (3), vertebral (1), sacral(1) iliac (5) fibula (1). All received CT, en-bloc surgery. Contaminated resection led to 45 grays irradiation: 2 sacral and 1 rib . In nov 04, 20/21 are EFS with a median FU of 153 months (range: 58 -227) 1 p. died (metastatic at 39 months). No local recurrence occured. Orthopedic results were excellent or good (18), fair (2), poor (1 amputated for infection of the prosthesis) following MSTclassification. 2 p. presented a colic adenocarcinoma, respectively 13 and 15 y following EW. They were 15 and 18 y old at diagnosis and had sacro-iliac locations irradiated post surgery. 8 were married, 3 had 6 normal children. 1 man was sterile (the same as colic cancer). All have a normal social life.

Conclusions:
this serie confirms the excellent outcome of children with EW (at the opposite of adults treated with the same scheme already published), the high value of early conservative carcinologic surger , the high risk of secondary radio-induced maligancy. This study emphasis the importance of systematic surgery in EW. Long term follow-up is mandatory to detect late recurrences or secondary maligancies.




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