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Key Points

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  1. As the second most frequent solid cancer of childhood, neuroblastoma remains an enigmatic tumor with a dismal outcome for advanced stage disease.

  2. Patient age and tumor stage at the time of diagnosis are the 2 most important variables contributing to neuroblastoma progression and relapse.

  3. Neuroblastoma stage is not only dependent on tumor location but also the presence of nodal and/or metastatic disease along with patient risk factors.

  4. The operative goal in neuroblastoma treatment is complete tumor removal of both the primary tumor and its adjacent involved lymph nodes (for stage I and II disease). There may also be considerable value in the cytoreductive removal of the maximum tumor burden that is both safe and feasible in more advanced stage disease.

  5. Surgical resection is not recommended in stage IV-S disease where the neuroblastoma is prone to spontaneous regression.

  6. Multimodal therapy-operation, chemotherapy and irradiation is key for neuroblastoma treatment depending on patient age, disease stage, response to therapy, and tumor relapse. The treatment goal remains both local control, metastatic control, and the prevention of relapse.

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Introduction

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Neuroblastomas exist as a spectrum of solid tumors that arise from primitive cells of fetal neural crest origin. These progenitor cells or neuroblasts localize along the sympathetic chain during embryological development and are destined to differentiate into nerve tissue cells as well as the tissue comprising the adrenal medulla. Neuroblastic tumors, therefore, can originate anywhere along the developmental pathway of the neural crest cell-derived sympathetic nervous system, including the adrenal medulla. Neuroblastoma accounts for 97% of all neuroblastic tumors. There is a wide spectrum of tumor phenotype, ranging from the benign differentiated form of ganglioneuroma to intermixed type of ganglioneuroblastomas to undifferentiated neuroblastoma. Clinically, they exhibit a broad range of tumor behavior that can span from a localized mass (which can undergo spontaneous regression), to an aggressive phenotype with disseminated disease involving distant organ metastases; and to disease relapse in the face of comprehensive multimodal treatment protocols. Accounting for 15% of all pediatric cancer-related deaths, neuroblastoma remains as one of the most difficult cancers to treat effectively for cure as reflected by the minimal gains made in overall survival after the treatment of high-risk disease. With research advances intensifying over the past decade, much has been discovered regarding the pathophysiology and molecular mechanisms underlying the tumorigenicity of neuroblastoma, and many novel therapies are currently in clinical trials. Yet in spite of this, neuroblastoma remains as an enigmatic cancer with an unpredictable clinical course and dismal overall outcome for advanced-stage disease.

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Epidemiology

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With between 600 and 700 new cases diagnosed in the United States each year, neuroblastoma is the third most common childhood cancer, trailing only leukemia and brain tumors. Responsible for approximately 10% of all childhood tumors, it is the most common extra-cranial solid tumor in the pediatric population. The clinical incidence in North America is roughly 1 in 10,000 live births, with ...

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