Introduction

Incidence and Management of Childhood Cancer

Every year 130–140 children per million under the age of 16 years, or around 1 out of 500 children, are diagnosed with childhood cancer. The incidence within the first 5 years of life is twice as high as from 6 to 15 years of age.

Survival probability has considerably changed within the past 30 years. Clinical research by cooperating groups of pediatric oncology centers has progressively increased the long-term survival rate from <20% before 1975 to >70–80% depending on the specific disease, in the new millennium.

International cooperation contributes to quality assurance because the majority of children with an oncologic disease are treated according to standard protocols. Reference centers therefore fulfill the important function of controlling, providing a second opinion and assessing the data of each child periodically.

Table 1 shows the average frequency of the different forms of pediatric neoplasia, based on international data.

While in adults about 80% of cancerous diseases pertain to the respiratory, gastrointestinal, and reproductive organs, only <5% of cancerous diseases of children are manifested in these organs. Furthermore, the histopathology of pediatric neoplasia differs markedly from that of adults: in children, embryonal and immature cells can be found at very different stages of development, which perpetually proliferate and rarely mature.

The variability within a particular childhood neoplasia and in the prognosis is high. Diagnosis and therapy must be adjusted to the individual child according to the clinical manifestation and the extent of the tumor.

Treatment normally requires 1–3 years, followed by check-ups for the following 3–7 years. The child newly diagnosed with cancer is critically ill during the first 2–6 months; after that his or her life continues similar to that of a healthy child, except that periodical treatment adjustment and check-ups are necessary. The initial treatment is carried out alternating between hospital care and care at home, the latter including the general practitioner and pediatrician as well as external nursing under the guidance of a pediatric cancer center.

Children with relapse need special attention and care. In particular, intensive treatments such as stem cell transplantation and experimental therapies yield hope.

Table 1 Overview of the frequency distribution of tumors in children and the incidence per year for children between 0 and 16 years of age

Last but not least, a child with a short life expectancy deserves high-quality palliative care by experienced professionals of the pediatric cancer team. Pediatric, clinical management may be divided as follows:

•Guidance/information of child and parents

•Therapy of complications and side effects (Chaps. 18 and 19)

•Specific therapy of the underlying disease, divided into induction of remission, consolidation, and maintenance

After confirmation of the diagnosis, an open discussion of all aspects between

the parents and the responsible physician should assure the following points:

•Close cooperation of child and family with the oncology team

•Explanation of diagnosis, prognosis, disease course, and treatment plan

•Stepwise orientation of therapy, including effect, side effects, and complications

•Emphasis of the aim to enable the child to lead as normal a life as possible

•To show critical openness in favor of the sick child if paramedical attendance or a second opinion is desired

•Attention to the healthiness of the whole family – the sister(s) and brother(s), the parents, grandparents, friends of the patient and others is recommended. It supports the patient’s management

•To determine how the information should be communicated to the young patient: in age-appropriate fashion, honestly, openly, in simple terms, and without frightening words; the child will want to hear the plan for the next days and weeks, look forward to the next festivity (birthday, Christmas, vacation); long-term prognosis are mainly of interest to the parents and other family members

For more guidance on how to deal with the patient, parents, and siblings, see Chap. 20.