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Soft tissue sarcoma of the head and neck in children and adolescents

✍ Scribed by Andrew T. Lyos; Helmuth Goepfert; Mario A. Luna; Norman Jaffe; Anais Malpica


Publisher
John Wiley and Sons
Year
1996
Tongue
English
Weight
810 KB
Volume
77
Category
Article
ISSN
0008-543X

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✦ Synopsis


BACKGROUND.

The experience of one institution in treating soft tissue sarcomas of the head and neck in a pediatric population is presented.

METHODS.

Case materials of 134 patients younger than 20 years who were referred to the University of Texas M. D. Anderson Cancer Center between 1970 and 1989 for treatment of sarcoma of the head and neck were retrospectively reviewed. Patients with rhabdomyosarcoma underwent multimodality treatment consisting of surgery, irradiation, and chemotherapy. Wide resection was the treatment used for patients with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). Adjuvant chemotherapy and irradiation were used to treat high grade neoplasms and residual disease. The clinical response to therapy was measured in terms of the disease-specific survival rate.

RESULTS.

Seventy-nine of 134 patients presented with untreated or biopsy-proven disease. Fifty-six had rhabdomyosarcoma and 23 had NRSTS. At 2 and 5 years, the disease-specific survival rates for patients with rhabdomyosarcoma were 74% and 63%, respectively, and patients with NRSTS had 80% and 75% disease-specific sunrival rates at 2 and 5 years, respectively.

CONCLUSIONS.

Rhabdomyosarcoma of the head and neck in children is effectively treated with multimodality therapy. Prognostic indicators for rhabdomyosarcoma include completeness of tumor resection and the development of recurrent disease. Aggressive surgical resection is the treatment of choice for patients with NRSTS. Prognostic indicators for NRSTS include completeness of tumor resection and the development of recurrent disease.


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## Abstract ## BACKGROUND Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft‐tissue sarcomas (STS) than the general population. The clinical findings and outcome in 43 children and adolescents with NF1 treated for STS in the Italian protocols between 1988 and