Indications for Radiotherapy and Chemotherapy in Malignant Tumors

Malignant peripheral nerve sheath tumors constitute around 5% of yearly incident soft tissue sarcomas in the United States, with around half of cases occurring in neurofibromatosis type 1, and prognosis remains poor despite multimodal therapy. Guidelines based on a high level of evidence for the use of adjunct radiotherapy and chemotherapy are lacking, but general recommendations on the most common indications can be made. In general, radiotherapy is employed as adjuvant treatment in intermediate- or high-grade tumors that are more than 5 cm in size or after marginal excision of low-grade lesions. Chemotherapy is mainly utilized in metastatic disease and in cases where preoperative chemotherapy may downstage tumors to improve surgical outcome. Targeted therapies are currently being investigated in clinical trials and constitute the main focus of future research endeavors aimed at prolonging survival. Multimodal therapy in specialized centers and a multidisciplinary approach remain crucial for achieving optimal outcome.