Neuroblastoma is the most common extracranial solid tumor in children.
About 70% of patients present with metastatic disease, with bone marrow and skeleton being the most common sites of metastases.
Patients may present with symptoms associated with adrenal masses, skeletal metastatic sites, ocular and skin involvement, neurological signs from paraspinal masses, etc. Additionally, constitutional symptoms with weight loss, anorexia, and fever are common with advanced disease stages.
Workup should include physical examinations, laboratory testing like urine cathecholamine homovanillic acid (HVA) and vanillylmandelic acid (VMA), bone marrow biopsy, radiological studies with abdominal computed tomography (CT) with contrast, metaiodobenzylguanidine (MIBG) scans, and Tc-99-diphosphonate bone scintigraphy.
Neuroblastoma staging has evolved from a surgical pathology–based to an image-based system.
Patients are divided into low-, intermediate-, and high-risk groups, based on their disease stage, tumor biology (MYCN amplification status, Shimada histology, and DNA ploidy), and age.
Chemotherapy plays a key role in the treatment of neuroblastoma, and its intensity increases from a few cycles of combination of drugs in low-risk cases to high-dose chemotherapy with stem cell rescue for high-risk group.
Surgical goals are based on disease risk groups: low-risk patients will need less aggressive resection, while intermediate- and high-risk patients should undergo gross total resection, when feasible.
Patients with low-risk disease usually have excellent outcomes with minimal therapy; the intermediate-risk group benefits from chemotherapy coupled with surgical tumor removal. High-risk group patients generally experience poor outcomes despite very intense treatment regimens.