Surgery in pediatric solid tumors with special reference to advanced neuroblastoma

Area of Science:

• Pediatric Oncology
• Cancer Genetics
• Surgical Oncology

Background:

• The role of surgery in neuroblastoma, particularly advanced or MYCN-amplified forms, remains controversial.
• Established surgical roles in Wilms' tumor and hepatoblastoma contrast with the uncertainty in high-risk neuroblastoma.
• MYCN amplification is a significant factor associated with poor prognosis in neuroblastoma.

Purpose of the Study:

• To analyze factors contributing to long-term survival in patients with MYCN-amplified neuroblastoma.
• To evaluate the impact of surgical radicality on survival outcomes.
• To identify essential components of multimodal therapy for advanced neuroblastoma.

Main Methods:

• Retrospective analysis of 66 patients with stage 3, 4, and 4S neuroblastoma and MYCN amplification treated between 1985 and 1993.
• Patients received induction chemotherapy regimens A1 or A3.
• Surgical resection of primary tumor and metastases, followed by autologous bone marrow or peripheral blood stem cell transplantation (ABMT/PBSCT).
• Surgical radicality categorized as total, subtotal, or partial.

Main Results:

• 19 out of 66 patients (29%) achieved long-term disease-free survival (>69 months).
• All long-term survivors, except one, received ABMT/PBSCT.
• Total surgical resection was achieved in 95% of long-term survivors, compared to 84% in those who died (p > 0.05).

Conclusions:

• Intensive chemotherapy, complete tumor resection, and timely ABMT/PBSCT are crucial for long-term survival in MYCN-amplified neuroblastoma.
• Multimodal therapy approach is essential for improving outcomes in this high-risk pediatric cancer.
• The study highlights the importance of radical surgery in conjunction with systemic therapy and stem cell support.