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KEY CONCEPTS

  • Patients with many common pediatric neoplasms, such as acute lymphoblastic leukemia (ALL), Wilms tumor, non-Hodgkin lymphoma, Hodgkin lymphoma, and germ cell tumors, have survival rates approaching or even exceeding 90%. However, progress has been limited for patients with some types of solid tumors, high-grade gliomas, and diffuse intrinsic brainstem gliomas.

  • Patients with ALL, the most common cancer in children, typically present with hepatomegaly or splenomegaly, pallor, fever, and/or bruising. ALL's genetic interrogation has enabled us to employ risk-based treatment strategies that have led to a cure rate greater than 98% in some ALL subtypes. Those with unfavorable cytogenetic factors, suboptimal responses to chemotherapy, or those who relapse require treatment with aggressive regimens and will benefit from novel treatment modalities.

  • Hodgkin lymphoma, the most common neoplasm among adolescents ages 15 to 19, commonly presents with painless supraclavicular or cervical adenopathy with or without the involvement of the mediastinum. Fever higher than 38 °C, drenching night sweats, and weight loss more than 10% of body weight within 6 months have been classified as “B symptoms” and affect staging and prognosis. Interim assessment with 18-fluorodeoxyglucose–positron emission tomography scan has allowed recognizing favorable responders who benefit from less toxic therapy.

  • In children, the central nervous system (CNS) neoplasms are mostly infratentorial. Patients present with increased intracranial pressure, which manifests as macrocephaly, vomiting, and developmental delay in infants and headache, vomiting, blurred vision, and altered mental status in older children. A gross total resection is the surgery goal, whenever it is safe and feasible. Chemotherapy and radiation therapy typically follow for the malignant CNS tumors except for chemoresistant tumors like ependymoma.

  • Non-neural solid tumors vary considerably by histology, tumor cell of origin, symptom spectrum, age at presentation, treatment, and outcome. The majority are treated with a multidisciplinary approach that includes surgical resection, radiation, and/or systemic chemotherapy. In some instances, like neuroblastoma and Wilms tumor, treatment is also determined by the patient's risk status, which incorporates biologic features.

  • In pediatric cancer survivors, long-term side effects of therapy include cardiovascular dysfunction in those exposed to anthracyclines and/or radiation, neurocognitive deficits, endocrinopathies, and secondary CNS tumors in patients who received radiation to the CNS, and breast and thyroid cancer for those exposed to radiation to the chest.

INTRODUCTION

Over the past five decades, the treatment of children and adolescents with cancer has improved dramatically.1 Patients with many common pediatric neoplasms, such as acute lymphoblastic leukemia (ALL), Wilms tumor (WT), non-Hodgkin lymphoma (NHL), Hodgkin lymphoma (HL), and germ cell tumors, have survival rates approaching or even exceeding 90%.2 However, progress has been limited for patients with some types of solid tumors, high-grade gliomas, and diffuse intrinsic brainstem gliomas. As in adults, pediatric patients who present with metastatic disease, who have a disease that does not respond to standard frontline therapies, and/or who have disease relapse face a grim prognosis despite intensive multimodal treatments that in survivors can cause long-term morbidity and disability.3 Thus, ...

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