Combined Treatment Strategy and Outcome of High Risk Neuroblastoma: Experience of the Children’s Cancer Hospital-Egypt

Emad Moussa, Mohamed Fawzy, Alaa Younis, Maged El Shafei, Mohamed Saad Zaghloul, Naglaa El Kinaai, Amal Refaat, Noha Atta, Alaa El Haddad

Department of Pathology, National Cancer Institute, Cairo University, and Children’s Cancer Hospital Egypt, Cairo, Egypt.
Department of Pediatric Oncology, Children’s Cancer Hospital-Egypt, Cairo, and Clinical Oncology Department, Faculty of Medicine, Menufeya University, Menufeya, Egypt.
Department of Pediatric Oncology, National Cancer Institute, Cairo University, and Children’s Cancer Hospital-Egypt, Cairo, Egypt.
Department of Radiodiagnosis, National Cancer Institute, Cairo University, and Children’s Cancer Hospital-Egypt, Cairo, Egypt.
Department of Radiotherapy, National Cancer Institute, Cairo University, and Children’s Cancer Hospital-Egypt, Cairo, Egypt.
Department of Research, Children’s Cancer Hospital-Egypt, Cairo, Egypt.
Department of Surgical Oncology, National Cancer Institute, Cairo University, and Children’s Cancer Hospital-Egypt, Cairo, Egypt.

DOI: 10.4236/jct.2013.49171                

Abstract

Background: Neuroblastoma (NB) is remarkable for its wide spectrum of clinical behavior and biological characteristics in relation to outcome. The use of aggressive therapy, including autologous hematopoietic stem cell transplantation (HSCT) and the addition of isoretionin (cis-Retinoic Acid/cis-RA), has increased survival rates of patients with advanced disease. Methods: Pediatric 271 newly diagnosed high risk NB patients were prospectively enrolled into the study. Patients received neoadjuvant chemotherapy of alternating cycles: [cyclophosphamide, doxorubicin, vincristine (CAdO)] and [etoposide, carboplatin]. Intensification courses of “ICE” (ifosfamide, carboplatin, and etoposide) regimen were administered to patients with bone marrow (BM) residual infiltration. Whenever safely feasible, complete surgical resection or debulking of the primary tumor was attempted for patients achieving partial response. Eligible patients underwent HSCT, while radiation therapy to the primary and metastatic sites, as well as maintenance with cis-RA was given for 6 months. Results: The median age of our patients was 2.8 years with male to female ratio of 1.65:1. At 4 years, the overall and event free survivals were 33.7% and 23.3% for the entire group under study, with significantly higher rates (42.7% and 35.6%, respectively) for HSCT patients (n = 94; p < 0.001). The outcome was also significantly correlated with response to induction therapy, pathological subtype, as well as other variables. Conclusion: Myeloablative therapy followed by stem cell rescue is regarded as the most important goal of high risk NB treatment to improve survival till present. Each of consolidation HSCT, post induction disease status, as well as international neuroblastoma pathology classification (INPC) subtype was an independent predictive variable of survival. A collaborative effort with an emphasis on biologic characteristics of aggressive disease and tailored therapy needs to be strengthened to further our understanding of this disease.

Keywords

High-Risk Neuroblastoma; Treatment; Outcome

Conflicts of Interest

The authors declare no conflicts of interest.

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