–Metastatic neuroblastoma (mainly infants and young children)

–Non-Hodgkin lymphoma

–Rhabdomyosarcoma

–Small-cell osteosarcoma

–Undifferentiated sarcoma

–Desmoplastic round-cell tumor

•Acute leukemia

14.11Treatment

•Therapeutic success reflected by multimodal therapy based on prospective randomized studies

•Risk-adapted individual therapy is the ultimate goal

•Close collaboration of the involved disciplines necessary

•The impressive improvement of clinical results is based on collaborative study groups, with the introduction of neoadjuvant and adjuvant chemotherapy in addition to surgery and judicious use of radiotherapy

•High-dose chemotherapy followed by autologous hematopoietic stem-cell therapy has not proved to improve outcomes

•Local therapy: intensive neoadjuvant chemotherapy followed by surgery, with the aim of complete resection when possible. Radiation therapy can be used when surgery is not indicated or possible in terms of achieving a complete resection or if it would be mutilizating

•Complete surgical resection is the primary aim. Patients treated with irradiation therapy alone are less likely to be cured than patients treated with surgery or surgery and radiotherapy

•EFT are radiosensitive. Irradiation therapy should be carefully evaluated and applied according to study protocols. Poor chemotherapy responders may benefit from radiotherapy

14.12Prognosis

•Dependent on surgical resection, tumor localization, tumor volume, presence of macroscopic metastases, molecular biological aspects

•Overall 5-year survival in the 1970s, 5–10%; currently (2004–2010) approximately 70%

14.12.1 Complications

•Relapse

•Adverse late sequelae include:

•Musculoskeletal abnormalities (surgery, radiotherapy)

•Secondary tumors

•Psychological and social problems