Adult life after surviving lymphoma in childhood.

INTRODUCTION: Almost all pediatric lymphomas are malignant, high-grade tumors. The combined incidence of Hodgkin's disease (HD) and non-Hodgkin lymphoma (NHL) reaches 10 to 12 new cases a year per million children under the age of 16 years, representing about 10% of all pediatric cancers. HD makes up to 40% and NHL 60% of pediatric lymphomas. During the last 20 years, cure rates raised dramatically so that currently over 90% of children and adolescents with HD and about 80% of those with NHL can be cured. As cure can be achieved in a large majority of patients, long-term effects and quality of life of the survivors are nowadays the principal challenges to pediatric oncologists. DISCUSSION: Like survivors from acute lymphoblastic leukemia, young adults cured from NHL may present with neurocognitive deficits, especially if treated at a young age and with cranial irradiation. Intrathecal or high-dose intravenous chemotherapy with methotrexate may induce the same problems, although in a lesser extent and severity. Large enough prospective cohort studies like the CCSS in the USA were able to show an increased risk of second malignant neoplasms, especially brain tumors in patients formerly treated with cranial irradiation. Reduced fertility can follow exposure to cyclophosphamide, especially in the male. Cardiac function must be serially evaluated over the long to very long-term because of potential cardiomyopathy after high anthracycline doses and/or mediastinal irradiation. Survivors from HD are at high risk of late complications. Radiation therapy to the neck and mediastinum (mantle field) induces a 50% risk of developing hypothyroidism and a 20% risk of developing thyroid nodules at 20 years. The risk of thyroid cancer is 18 times higher the expected rate for the general population. Secondary aggressive breast cancer shows a cumulative risk of 30% at 30 years after radiotherapy. Other structures affected by mediastinal irradiation are the heart (pericardial, myocardial and endocardial structures), the great arteries (fibrosis, stenosis, aneurysms) and the central portion of the lungs (diffusion troubles, restrictive pneumopathy). Cardiac toxicity can be enhanced by the concomitant therapy with adriamycin and lung toxicity by bleomycin. Radiotherapy to the paraaortic and iliacal lymph nodes can affect gonadal function both in males and females; concomitant chemotherapy with alkylating agents like cyclophosphamide and especially procarbazine have a synergistic action and can lead to premature menopause as well as infertility. Although the vast majority of survivors from pediatric lymphomas fare well, a minority present with extreme symptoms of depression and psychosomatic distress; female sex, low socio-economic status and treatment with intensive chemotherapy are important risk factors for a poor psychosocial outcome. CONCLUSION: It is therefore crucial, but not always easy, to inform patients and families about potential late effects and organize follow-up after the pediatric age. A well functioning network of pediatric oncologists, GP's, adult oncologists and other specialists of adult medicine must be developed in order to prevent, early detect and treat expected long-term toxicities

The Swiss Childhood Cancer Registry: rationale, organisation and results for the years 2001-2005.

Childhood cancer is a rare but severe disease. Therefore central registration of all cases is essential for surveillance and management. This paper describes the methodology and basic results of the Swiss Childhood Cancer Registry (SCCR). The SCCR was established in 1976, originally as a national hospital-based registry of childhood malignancies. All 9 paediatric oncology-haematology clinics in Switzerland provide baseline and follow-up information on all children diagnosed with cancer. These data are registered centrally and diagnoses are coded according to the International Classification of Childhood Cancer. From 2001-2005, 887 cases of childhood cancer in Swiss residents under the age of 15 years were registered in the SCCR. Of these, 281 (31.7%) were leukaemias, 223 (24.0%) were CNS tumours, and 116 (13.1%) were lymphomas. The age-standardised annual incidence per 1 Million person-years (age below 15 years; world standardisation) was 154.0 (95% CI 143.7-164.3; N = 887). The incidence was higher for boys (170.2, 155.0-185.4; N = 501) than for girls (136.9, 123.0-150.8; N = 386). The close collaboration between all paediatric oncologists-haematologists in Switzerland and a university department allowed the creation of a national population-based cancer registry with detailed clinical information. The SCCR produces cancer type specific incidence and survival estimates and allows the development of nested research projects on childhood cancer aetiology, management and outcome, both on a national and on an international level

Physicians’ experience with follow-up care of childhood cancer survivors – Challenges and needs

BACKGROUND: Regular follow-up care is essential for childhood cancer survivors, but we know little about physicians’ experience with it. We aimed to describe: (1) involvement of Swiss physicians in follow-up care; (2) content of follow-up care provided; (3) problems encountered; and (4) additional resources needed. MATERIALS AND METHODS: Within this cross-sectional survey we sent adapted questionnaires via professional associations to a sample of medical oncologists (MOs), paediatric oncologists (POs), general practitioners (GPs) and paediatricians (P) in Switzerland. Only oncologists involved in follow-up care were asked to report problems. GPs and Ps not involved in follow-up could indicate why. All physicians were asked about the content of follow-up care provided and additional resources needed. RESULTS: A total of 183 physicians responded (27 MO, 13 PO, 122 GP, 21 P). Involved in follow-up were 81% of MOs, 85% of POs, 39% of GPs and 81% of Ps. Follow-up content differed between oncologists (MO and PO) and generalists (GP and P), with generalists examining or informing less in regard to the former cancer. POs reported more problems than MOs: many POs reported problems with transition of survivors to adult care (91%), and because of financial resources (73%) and time restraints (73%). MOs reported most problems during transition (23%). Not being aware of a survivor was the most common reason for GPs and Ps not participating in followup (74%). All groups reported a need for standardised protocols (85–91%) and specialised training (55–73%). GPs (94%) and Ps (100%) additionally desired more support from oncologists. CONCLUSIONS: To improve quality and efficiency of follow-up care a national follow-up care model including standardised protocols and guidelines needs to be developed

No evidence of response bias in a populationbased childhood cancer survivor questionnaire survey-Results from the Swiss Childhood Cancer Survivor Study

Purpose This is the first study to quantify potential nonresponse bias in a childhood cancer survivor questionnaire survey. We describe early and late responders and nonresponders, and estimate nonresponse bias in a nationwide questionnaire survey of survivors. Methods In the Swiss Childhood Cancer Survivor Study, we compared characteristics of early responders (who answered an initial questionnaire), late responders (who answered after ≥1 reminder) and nonresponders. Sociodemographic and cancer-related information was available for the whole population from the Swiss Childhood Cancer Registry. We compared observed prevalence of typical outcomes in responders to the expected prevalence in a complete (100% response) representative population we constructed in order to estimate the effect of nonresponse bias. We constructed the complete population using inverse probability of participation weights. Results Of 2328 survivors, 930 returned the initial questionnaire (40%); 671 returned the questionnaire after ≥1reminder (29%). Compared to early and late responders, we found that the 727 nonresponders (31%) were more likely male, aged <20 years, French or Italian speaking, of foreign nationality, diagnosed with lymphoma or a CNS or germ cell tumor, and treated only with surgery. But observed prevalence of typical estimates (somatic health, medical care, mental health, health behaviors) was similar among the sample of early responders (40%), all responders (69%), and the complete representative population (100%). In this survey, nonresponse bias did not seem to influence observed prevalence estimates. Conclusion Nonresponse bias may play only a minor role in childhood cancer survivor studies, suggesting that results can be generalized to the whole population of such cancer survivors and applied in clinical practice

No evidence of response bias in a population-based childhood cancer survivor questionnaire survey - Results from the Swiss Childhood Cancer Survivor Study.

PURPOSE This is the first study to quantify potential nonresponse bias in a childhood cancer survivor questionnaire survey. We describe early and late responders and nonresponders, and estimate nonresponse bias in a nationwide questionnaire survey of survivors. METHODS In the Swiss Childhood Cancer Survivor Study, we compared characteristics of early responders (who answered an initial questionnaire), late responders (who answered after ≥1 reminder) and nonresponders. Sociodemographic and cancer-related information was available for the whole population from the Swiss Childhood Cancer Registry. We compared observed prevalence of typical outcomes in responders to the expected prevalence in a complete (100% response) representative population we constructed in order to estimate the effect of nonresponse bias. We constructed the complete population using inverse probability of participation weights. RESULTS Of 2328 survivors, 930 returned the initial questionnaire (40%); 671 returned the questionnaire after ≥1reminder (29%). Compared to early and late responders, we found that the 727 nonresponders (31%) were more likely male, aged <20 years, French or Italian speaking, of foreign nationality, diagnosed with lymphoma or a CNS or germ cell tumor, and treated only with surgery. But observed prevalence of typical estimates (somatic health, medical care, mental health, health behaviors) was similar among the sample of early responders (40%), all responders (69%), and the complete representative population (100%). In this survey, nonresponse bias did not seem to influence observed prevalence estimates. CONCLUSION Nonresponse bias may play only a minor role in childhood cancer survivor studies, suggesting that results can be generalized to the whole population of such cancer survivors and applied in clinical practice

Hémato-oncologie pédiatrique: de la biologie cellulaire aux traitements ciblés et individualisés [Pediatric hemato-oncology: from cellular biology to individualized targeted therapies].

Progresses in pediatric oncology over the last decades have been dramatic and allow current cure rates above 80%. There are mainly due to multicentre clinical trials aiming at optimizing chemotherapy protocols as well as local therapies in a stepwise approach. Most of the new anticancer drugs currently in development are based on targeted therapies, directed to specific targets present only in or on tumor cells, like growth factor receptors, mechanisms involved in proliferation, DNA repair, apoptosis, tumor invasion or angiogenesis. Concerning bone marrow transplantation also, new strategic approaches are in advanced development. They aim at reducing treatment induced toxicity and enhancing efficacy at the same time. This short paper would like to point out these new technologies, which should be known by the general practitioner