Medical Overview

Many aspects of oncology are different for children and adolescents than for adults. Differences include not only the types of malignancies that are most common in each age group, the prognoses, and the indicated treatments but also the types of settings in which cancer is treated, the types of services available at these centers, and the use of cancer protocols (American Academy of Pediatrics 2009). Although the rate of childhood cancer diagnosis in the United States has been increasing slightly (0.6% per year) over the past three decades, it is still only approximately 15 cases per 100,000 (American Cancer Society 2009). Cancer incidence rates in children ages 0-19 years, by sex, are listed in Table 15-1. In 2009, it is estimated that approximately 10,730 new diagnoses of cancer will be made in children 0-14 years of age.

In general, the prognosis for pediatric malignancies is better than for adult malignancies and is far better than it was 40 years ago. Before the 1970s, the 5-year relative survival rate for all childhood cancers combined was less than 50%; today, it is 80% (American Cancer Society 2009). This improved rate is largely due to increased understanding of cancer biology and of pharmacogenetics, leading to more targeted and effective treatment.

For children, as for adults, significant differences in outcome exist for different sites and histological types of cancers (see Table 15-2). Solid tumors, such as Wilms' tumor, are rarely fatal and often require lit tle more than surgical removal of the affected kidney. Other tumors or hematological malignancies may require years of intensive treatment, with resulting tox-icities. The various cancers of the white blood cells, known collectively as leukemia, are the most common type of malignancy in children younger than 14 years. Leukemia accounts for approximately 33% of all childhood cancers and for one-third of cancer deaths in children younger than 14 years. Cancers in the brain or nervous system are the second most common form of cancer in children and the second leading cause of cancer deaths in children younger than 14 years (American Cancer Society 2009).

The American Academy of Pediatrics (2009) has recommended that all children with cancer be treated at a major cancer treatment center and specified the medical, surgical, and psychosocial resources that should be available at such centers. These include pediatric social workers, pediatric psychologists, child life specialists, and family support group services. Specific treatment protocols, designed by national pediatric oncology groups, are used, with most children entered into clinical trials so that the data can be used to evaluate the effectiveness and toxicity of specific treatment protocols (Reaman 2009; Ruccione et al. 2005). Long-term follow-up focuses on both quality of life and survival. The goal is to minimize short- and long-term toxicity while maximizing long-term survival. This focus on survivorship issues is exemplified in the development of specific guidelines for care of children after treatment ends (Children's Oncology Group 2008).

Table 15-1. Cancer incidence ratesa in U.S. children ages 0-19 years by sex, 2002-2006

Site

Male

Female

Total

All sites

17.7

15.5

16.6

Leukemia

5.1

3.9

4.5

Acute lymphocytic

3.9

2.9

3.4

Brain and other nervous

3.1

2.7

2.9

Soft tissue

1.1

1.0

1.1

Non-Hodgkin's lymphoma

1.4

0.8

1.1

Kidney and renal pelvis

0.6

0.7

0.6

Bone and joint

1.0

0.8

0.9

Hodgkin's lymphoma

1.2

1.1

1.2

aPer 100,000, age-adjusted to the 2000 U.S. standard population.

Source. Adapted from Horner MJ, Ries LAG, Krapcho M, et al. (eds): SEER Cancer Statistics Review, 1975-2006. Bethesda, MD, National Cancer Institute, http://seer.cancer.gov/csr/1975_2006/, based on November 2008 Surveillance, Epidemiology and End Results (SEER) data submission, posted to the SEER Web site, 2009.

aPer 100,000, age-adjusted to the 2000 U.S. standard population.

Source. Adapted from Horner MJ, Ries LAG, Krapcho M, et al. (eds): SEER Cancer Statistics Review, 1975-2006. Bethesda, MD, National Cancer Institute, http://seer.cancer.gov/csr/1975_2006/, based on November 2008 Surveillance, Epidemiology and End Results (SEER) data submission, posted to the SEER Web site, 2009.

Genetic susceptibility plays a part in some pedi-atric malignancies, such as retinoblastoma, generally through a mutation in tumor suppressor genes (Plon and Nathanson 2005). Specific clinical surveillance can now be used to improve survival and quality of life for at-risk children (Rao et al. 2008).

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