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Rhabdomyosarcoma and other soft tissue sarcomas of childhood
1St. Jude Children's Research Hospital, Memphis, Tennessee.
Abstract:
Often treated as a single clinical entity, the pediatric soft tissue sarcomas include neoplasms of diverse histology that can originate from any anatomic site and exhibit varied patterns of local spread and metastasis. Recent developments with potential clinical impact include new diagnostic and prognostic markers stemming from advances in molecular biology, and increasing concern for the late effects of therapy in a growing population of long-term survivors. Initial clinical data indicate the value of MyoD1 protein expression in classifying previously indeterminate primitive undifferentiated tumors. The reported sensitivity and specificity of P-glycoprotein expression for the identification of chemoresistant disease in pediatric sarcomas should spawn studies to clarify its prognostic value and potential therapeutic strategies to circumvent the multidrug-resistance phenotype. Tumor cell DNA ploidy may also have prognostic and diagnostic value. An abundant interest in alleviating the late effects of adjuvant therapy and surgery is reflected in many clinical reports by oncologists, surgeons, radiotherapists, ophthalmologists, and other specialists.