BACKGROUND: Osteosarcoma is the most common malignant bone tumor in children and young people. Efficacy of multiagent MAP (methotrexate, doxorubicin [Adriamycin], cisplatin) chemotherapy may be influenced by multiple cellular pathways. This pilot study aimed to investigate the association of 36 candidate genetic polymorphisms in MAP pathway genes with histological response, survival, and grade 3‐4 chemotherapy toxicity in osteosarcoma. METHODS: Blood samples were obtained from 60 patients who had completed MAP chemotherapy. All patients were manually genotyped for 5 polymorphisms. The remaining 31 polymorphisms were genotyped in 50 patients using the Illumina 610‐Quad microarray. Associations between candidate polymorphisms and histological response, progression‐free survival, and toxicity were estimated using Pearson chi‐square and Fisher exact tests, the Kaplan‐Meier method, the log‐rank test, and the Cox proportional hazards model. RESULTS: Poor histological response was increased in variants of ABCC2 c.24C>T (P = .011) and GSTP1 c.313A>G p.Ile105Val (P = .009), whereas MTHFD1 c.1958G>A p.Arg653Gln was protective (P = .03). Methotrexate toxicity was increased in variants of MTHFR c.1298A>C p.Glu429Ala (P = .038), ABCB1 c.3435T>C Ile145Ile (P = .027), and ABCC2 c.3563T>A p.Val1188Glu (P = .028). Variants of GSTP1 c.313A>G p.Ile105Val were at increased risk of myelosuppression (P = .024) and cardiac damage (P = .008). CONCLUSIONS: This pilot study represents the most comprehensive study to date examining the role of genetic polymorphisms in osteosarcoma. Although small and retrospective, it shows that several polymorphisms appear to significantly influence toxicity and clinical outcome. These deserve prospective validation in the hope of optimizing treatment for resistant disease and reducing the late effects burden. Cancer 2012. © 2011 American Cancer Society.

The Children's Cancer and Leukaemia Group recommendation that children with PTCL-u receive group T therapy is supported by this series. The numbers of children with other types of PTCL were too small to allow conclusions on best therapy.

Purpose: To facilitate the implementation of long-term follow-up (LTFU) care and improve equality of care for childhood, adolescent, and young adult (CAYA) cancer survivors, the PanCareSurFup Guidelines Working Group developed evidence-based recommendations for the organisation of LTFU. Methods: We established an international multidisciplinary guideline panel. A systematic review of the literature published from 1999-2017 was completed to answer six clinical questions. The guideline panel reviewed the identified studies, developed evidence summaries, appraised the quality of the body of evidence and formulated recommendations based on the evidence, expert opinions, and the need to maintain flexibility of application across different healthcare systems. Results: We provide strong recommendations based on low level evidence and expert opinions, regarding organization of LTFU care, personnel involved in LTFU care, components of LTFU care and start of LTFU care. We recommend that risk adapted LTFU care provided under the guidance of a cancer survivorship expert service or cancer centre should be available and accessible for all CAYA cancer survivors throughout their lifespan. Conclusion: Despite the weak levels of evidence, successful and effective implementation of these recommendations should improve LTFU, thereby leading to better access to appropriate healthcare services and an improvement in health outcomes for CAYA cancer survivors. Implications for cancer survivors: To improve health outcomes and quality of survivorship of current and future survivors, continued age-adapted education of survivors about the cancer, its treatment, risk for late-effects, importance of health behaviours, and necessity of LTFU is important along the cancer and survivorship trajectory.

Background. Previous reviews of outcomes in specific sarcoma populations suggest patients have poor quality of life. In most of these reviews, there is a predominant focus on physical function rather than psychosocial outcome. The aim of this review was to describe the psychosocial impact of diagnosis and treatment on patients with all types of sarcoma. Methods. Searches were conducted through six electronic databases for publications of any study design using a validated patient-reported outcome measure reporting the psychosocial impact in this population. Results. Eighty-two studies fulfilled the inclusion criteria. Most (65%) were assessed of being of reasonable quality. The most common aspect of psychosocial wellbeing measured was quality of life (80%). Due to the heterogeneity of methods, outcomes, and populations, it was not possible to make definitive conclusions. It seems there is an improvement in the physical aspects of quality of life over time but not in psychosocial function or mental health. There was no change in mental health scores, but patients reported an improvement in adjusting to normal life. There are no differences according to the type of surgery patients receive, and psychosocial outcomes tend to be poorer than the general population. There is no consistency in identifying the factors that predict/influence psychosocial wellbeing. Conclusion. The published literature does not provide a clear understanding of the impact of sarcoma diagnosis and treatment on psychosocial wellbeing. Instead, the review demonstrates a need for well-designed studies in this area and a more consistent approach to the measurement of patient-reported outcomes, which include psychosocial domains. Recommendations for future research have been proposed.

ObjectiveThe aim of this study is to explore the experiences of patients with primary bone cancer.DesignQualitative study design using semistructured interviews and focus groups.SettingHospitals across the UK and recruitment through UK sarcoma charities and support groups.MethodsSemistructured telephone/face-to-face interviews and focus groups with a purposive sample of 26 participants. Data were analysed using Framework Analysis.ParticipantsPatients (n=26) with primary bone cancer aged 13–77 years. The majority were male (69%), white (85%); diagnosed within 4 years (54%); and had lower limb sarcoma (65%). Ten participants had undergone an upper/lower limb amputation (39%).ResultsThe health-related quality-of-life domains of physical, emotional and social well-being and healthcare professionals’ role were the overarching themes of analysis. The physical domain anchored patient experiences. The intensity and length of treatment, the severity of side-effects, the level of disability after surgery and the uncertainty of their prognosis had an impact on patient’s self-image, confidence, mood and identity, and caused disruption to various aspects of the patients’ social life, including their relationships (emotional and sexual) and participation in work/school and leisure activities. Adaptation was influenced by the way patients dealt with stress and adversity, with some finding a new outlook in life, and others struggling with finding their ‘new normal’. Family and friends were the main source of support. Healthcare professional’s expertise and support was critical. Rehabilitation services had a considerable role in patient’s physical and emotional well-being, but inequitable access to these services was apparent.ConclusionsThis study described the impact of primary bone cancer on patients’ well-being and adjustment over time with the identification of influencing factors of better/worse experiences. It showed that impact was felt after end of treatment and affected patients at different life stages. Holistic models of survivorship care are needed.