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6.P.09 HEAD AND NECK SOFT TISSUE SARCOMAS IN CHILDREN AND ADOLESCENTS. EXPERIENCE FROM ONE INSTITUTION



Abstract

Beckground: Head and neck region is a rare site location for sarcomas and there are difficulties of surgery management.With the exception of those arising in relatively superficial locations, are rarely amenable to wide local excision. Incisional biopsy for diagnostic purposes is usually all that is feasible. Multimodal treatment is mandatory to achieve local control.

The present study examines multimodal treatment outcome in children and adolescents with head and neck soft tissue sarcomas (H& NS).

Patients and Methods. Patients with H& NS who underwent chemotherapy +/− radiotherapy, after surgery, in Institute of Oncology Bucharest between 1990– 2007 were identified. Clinical charts and pathology reports were examinated.

The study included 42 pts.(29 male and 13 female); median age was 14 years years (range 3 – 21)

Sites of primary tumor: parameningeal: 18 pts; non-parameningeal 16 pts and orbit: 8pts. Histologic types: rhabdomyosarcomas and undifferentiated sarcomas represent 52%. Staging (TNM pretreatment staging classification for IRS-IV): St. I+II-36% ; St. III- 34%; St.IV-30%

All the pts were treated multidisciplinary: Surgery + Chemotherapy(PCT) +/− Radiotherapy(RT). Type of surgery performed: partial excision:54%; complete excision: 4 pts (9%); incisional biopsy: 37%. Type of RT: Exclusive RT in inoperable tumors(4 pts) ; external RT- postoperative(59%) curietherapy: 1 pt; gammaknife: 1pt. Neoadjuvant PCT were applied for 16% of pts and adjuvant PCT for 85,7% of pts.. Chemotherapy protocols used after 1992 was: IVA, IVE, VAIA, CEVAIE, and before 1992: CYVADIC, CYVADACT. All cases were followed minimum 2 years after the end of the treatment.

Results: Overall survival estimated by Kaplan Meier curve: 66%/ 1 year, 43%/ 5 years. OS according to the stages of disease ; stage I-II: 93% at 1 year,73% ar 5 years; stage III and IV: 47% at 1 year, 23% at 5 years.

EFS in sarcomas treated with S+PCT+RT is 41%, versus 16.5% for sarcomas treated with S+CT.

Observations: The diagnosis in advanced stages was due to the confusion with nononcological diseases.

Conclusions: 1. EFS was highest in sarcomas treated S+CT+RT face to sarcomas trated S+CT. 2 EFS at 2–3 yrs. in children’s soft tissues sarcoma could be considered as cure.

Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de