Problems and controversies in the management of childhood sarcomas.

ABSTRACT

Multidisciplinary care and advances in chemotherapy have dramatically improved the prognosis of paediatric sarcoma patients, but much work remains. There are new techniques for molecular diagnosis of Ewing's sarcomas and alveolar rhabdomyosarcomas, with molecular techniques of staging and subclassification under development. Osteosarcoma is a clinically heterogeneous disease which continues to resist biologic diagnosis, classification, or staging. In chemotherapy, the roles of ifosfamide and doxorubicin in rhabdomyosarcoma treatment remain unclear. While many children with metastatic or recurrent sarcomas undergo massive therapy with peripheral stem cell or autologous marrow rescue, the efficacy of these manoeuvres is debatable. Osteosarcoma appears to respond best to regimens containing doxorubicin and cisplatin, while the roles of alkylating agents, high-dose methotrexate, and carboplatin remain unclear. Ewing's sarcoma treatment increasingly includes surgery because of the risk of secondary osteosarcoma after radiation. Most osteosarcoma patients now have limb-sparing excisions, though amputation may provide better function and cosmesis with less risk of complications in some patients. The role of multimodal treatment including chemotherapy for the miscellaneous soft tissue sarcomas remains uncertain.