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This chapter reviews common childhood hematologic and oncologic conditions, and discusses principles of ED management of initial presentations and the complications of established disease.

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Most children with cancer are treated within the paradigm of a research protocol. Collectively, pediatric cancers impose a disproportionate burden upon the U.S. health care system; approximately 12,400 neoplasms are diagnosed each year in children under the age of 20 years old. Individually, with a few exceptions, each malignancy is quite rare, challenging researchers to develop safe and effective therapies. In 1998, the major North American pediatric cancer clinical trials groups (the Children’s Cancer Group, the Pediatric Oncology Group, the National Wilms’ Tumor Study Group, and the Intergroup Rhabdomyosarcoma Study Group) merged to form the Children’s Oncology Group (COG). As the largest childhood cancer research organization in the world, the COG is composed of >200 pediatric cancer programs in North America, Switzerland, the Netherlands, New Zealand, and Australia. Funded by the National Cancer Institute, the COG is instrumental in coordinating efforts that translate laboratory investigations to clinical trials to standard of care.

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Childhood cancer is second only to major trauma as a cause of death in children >1 year old. The majority of pediatric cancers are highly sensitive to radiation and chemotherapy, so long-term survival rates are significantly better than in adults: around 70% overall.1

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Even as cure rates for most childhood cancers have continued to improve2 [particularly in lymphoma and acute lymphoblastic leukemia (ALL)], the incidence of new diagnoses has also risen. The most common childhood malignancies are discussed below. Each diagnosis is organized to first address the initial diagnosis and care of previously undiagnosed cancer, and then to address specific complications and treatment. A specific section addresses the infectious, metabolic, and structural complications commonly seen in a variety of childhood cancers.

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Epidemiology

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Acute leukemias, including ALL and acute myelogenous leukemia (AML), are the most common cancers in children, accounting for a third of all malignancies. ALL accounts for approximately three fourths of pediatric leukemias and, if diagnosed early, carries an extremely high cure rate. Remission rates may be as high as 98%, and 5-year survival is in the 75% to 80% range.3 The peak incidence of ALL is 3 to 5 years of age, with younger children having the best outcomes.3,4 Prior exposure to chemotherapy and radiation in the treatment of other childhood cancers has caused an increase in the incidence of secondary AML.

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AML, despite the name, may arise from many different precursors (myeloid, erythroid, megakaryocytic, monocytic). This heterogenous group of leukemias is thought to arise from multiple genetic mutations that allow both unchecked proliferation and impaired differentiation. The age-related incidence of AML is relatively uniform throughout childhood.

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Patients being treated for AML have a higher incidence of complications than those with ALL, especially infections. This is primarily due to the greater intensity of ...

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