The treatment section of this document is organized to correspond with the Children's Oncology Group (COG) risk-based schema for the treatment of neuroblastoma. This schema is based on three factors: patient age at diagnosis, certain biological characteristics of the patient's neuroblastoma tumor, and the stage of the tumor as defined by the International Neuroblastoma Staging System (INSS). The INSS has replaced the previously used Children's Cancer Group (CCG) and Pediatric Oncology Group (POG) staging systems. The INSS is described below, and the COG risk-based treatment schema is described in Table 1 in this section.
A thorough evaluation for metastatic disease should be performed prior to therapy initiation. The following investigations are recommended:
- Bone marrow should be assessed by bilateral posterior iliac crest marrow aspirates and trephine (core) bone marrow biopsies to exclude bone marrow involvement. To be considered adequate, core biopsy specimens must contain at least 1 cm of marrow, excluding cartilage. Bone marrow sampling may not be necessary for tumors that are otherwise stage 1.
- Before resection of the primary tumor, bone should be assessed by metaiodobenzylguanidine (MIBG) scan, which is applicable to all sites of disease, and by technetium 99 scan if the results of the MIBG scan are negative or unavailable. Imaging with 123 I-MIBG is optimal for identifying soft tissue and bony metastases and is superior to 18F-FDG positron emission tomography/computerized tomography (PET/CT) in a prospective comparison. Additional imaging of isolated or equivocal positive lesions is recommended if the primary tumor does not take up MIBG.
- Palpable lymph nodes should be clinically examined and histologically confirmed if indicated for staging.
- The abdomen and liver should be assessed by CT scan and/or magnetic resonance imaging (MRI). Ultrasound is considered suboptimal for accurate 3D measurements. If extension of abdominal disease or pulmonary metastasis is suspected, the chest should be examined by CT scan.
- Lumbar puncture should be avoided as central nervous system (CNS) metastasis at diagnosis is rare, and lumbar puncture may be associated with an increased incidence of subsequent development of CNS metastasis.
- Paraspinal tumors may extend through neural foramina to compress the spinal cord. Therefore, MRI of the spine adjacent to any paraspinal tumor is recommended.