[PNET/Ewing tumours: current treatments and future perspectives].

Area of Science:

• Oncology
• Pediatric Oncology
• Skeletal Oncology

Context:

• Ewing tumours are a rare but aggressive bone cancer primarily affecting adolescents and young adults.
• They are characterized by small, round, blue malignant cells with potential neural differentiation.
• Ewing sarcoma is the second most common primary bone malignancy in this age group.

Purpose:

• To review the biological and therapeutic aspects of Ewing tumours.
• To summarize advancements in chemotherapy, surgery, and radiotherapy for Ewing sarcoma.
• To highlight prognostic factors and the need for novel therapeutic agents for metastatic disease.

Summary:

• Chemotherapy has significantly improved survival rates for localized Ewing tumours (65-70%) and metastatic disease (25-30%).
• Surgical intervention and advanced imaging techniques are crucial for treatment indication and optimization.
• Radiotherapy, including advanced techniques like IMRT, offers benefits but long-term toxicities remain a concern.

Impact:

• Identified clinical and biological prognostic factors guide therapeutic choices.
• The review underscores the poor prognosis of metastatic Ewing tumours, necessitating new drug development.
• This 2009 review provides a comprehensive overview of Ewing sarcoma's status in biology and treatment.