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Disease summaries

Core principles for comprehensive quality cancer care for children and adolescents

Outcomes for children and adolescents with cancer are markedly influenced by comprehensive and accurate initial evaluation as well as treatment. Modern risk-based therapy often depends upon biologic testing of diagnostic material. Therefore all children and adolescents suspected of having cancer must have access and timely referral to pediatric oncology specialists for high quality screening and diagnostic testing, treatment, rehabilitation and post-treatment monitoring for recurrence as well as long-term and late effects of therapy.

Thalassemia major

Thalassemia major is the most common life-threatening genetic disease. Even though long-term supportive care with regular red cell transfusion and intensive chelation therapy for iron overload may prolong life-expectancy to the fourth decade of life, bone marrow transplantation remains the only curative option. Appropriate supportive care is quite expensive and may not be regularly available in most low-income areas. Without transfusion support children rarely survive beyond 5 years and with transfusions but no chelation therapy life expectancy rarely extends beyond adolescence. The overall costs of long term supportive care are well above those of transplantation, this trend will most likely increasing over time as chelation therapy becomes more expensive while transplant procedures tend to become simpler and more tolerable.

Rhabdomyosarcoma (RMS)

Accounts for 50% of soft tissue sarcomas in the pediatric age group. The estimated average annual incident rate is 4.3 cases per million children, 2/3 are less than 6 years old and the remaining third are adolescents. It is slightly more frequent in males1.