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Key Points

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  1. Cancer accounts for 10% of all pediatric deaths.

  2. Surgically treated cancers, neuroblastoma, the Wilms tumor, rhabdomyosarcoma, and hepatoblastoma, collectively account for about 20% of all pediatric cancers.

  3. Children typically tolerate chemotherapy better than adults.

  4. Pediatric cancer therapy is associated with several long-term morbidities, including neurologic deficits, cardiac illness, and the development of secondary malignancies.

  5. Neuroblastoma may be treated with surgery alone; surgery plus chemotherapy (carboplatin, cyclophosphamide, doxorubicin, and etoposide), or surgery, chemotherapy and hematopoietic stem cell transplantation, isotretinoin, and a monoclonal GD2 antibody depending on the stage.

  6. Most patients with the Wilms tumor receive vincristine and dactinomycin with additional agents and radiation added for higher-risk tumors.

  7. Vincristine, dactinomycin, and cyclophosphamide, along with radiation for local control, represent the mainstays of therapy for pediatric rhabdomyosarcoma.

  8. The majority of hepatoblastomas are treated with neoadjuvant chemotherapy (consisting of combinations of cisplatin, doxorubicin, vincristine, and 5-fluorouracil) to allow for surgical resection and cure.

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Introduction

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Survival of pediatric malignancies has improved dramatically over the past half-century due to advances in the safety and efficacy of chemotherapeutic agents and radiation therapy. Additionally, although strategies to limit toxicity have likewise been developed, cancer therapy during childhood can be associated with lifelong morbidity. Chemotherapy and radiation serve as adjuncts to surgery in the treatment of neuroblastoma, the Wilms tumor, rhabdomyosarcoma, and hepatoblastoma; optimization of these regimens is the subject of ongoing clinical trials. A basic understanding of these topics is essential for all providers who care for the child with cancer.

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Epidemiology and Survival Statistics for Pediatric Cancers

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The incidence of cancer in children is relatively low, 133 cases per million children under age 15 years, compared with that of adults, 4620 cases per million, in the United States. The incidence bimodal, with an incidence rate of 200 cases per million in children age 2 years or younger, and a second peak during adolescence. Before age 2 years, central nervous system (CNS) malignancies, neuroblastoma, acute myeloid leukemia (AML), the Wilms tumor, and retinoblastoma account for the majority of diagnoses, while Hodgkin lymphoma, osteosarcoma, and the Ewing sarcoma become more prevalent during adolescence. Overall, the most common cancers in children under 15 years are acute lymphoblastic leukemia (ALL, 24.5% of cases), CNS tumors (20.2%), neuroblastoma (7.5%), and the Wilms tumor (6.1%) (Table 87-1). In contrast to adult tumors, less than 10% of pediatric cancers are epithelial in origin.

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Table Graphic Jump Location
Table 87-1   Distribution of Cancer Types in Children Less Than age 15 Years 

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