Elements of successful patient involvement in clinical cancer trials: a review of the literature.

Patient involvement in clinical cancer research has gained much ground in the past few years and studies demonstrated positive outcomes of such involvement. Yet, they also indicated a lack of evidence around best methods and practices to achieve successful patient involvement. The aim of this literature review was to provide a synthesis of elements contributing to successful and meaningful ways of involving patients in oncology trials across different stages of research. This synthesis can offer practical support to researchers in their patient involvement journey. A PubMed literature search for original articles published between 2012 and early 2023 was carried out. In total, 3132 articles were identified, among which 152 were fully assessed for eligibility. Thirty-three articles met the predefined inclusion criteria and were subjected to a quality checklist. Patient involvement occurred most often in the development stage of cancer trials (85%) and was continuous and integrated throughout the entire lifecycle of research (67%). In total, 58 elements of successful patient involvement were identified, such as clearly defined roles and responsibilities of patient partners, input of multiple patients to ensure diversity, and regular touchpoints in the project. All these elements can be applied in future studies from the planning stage to the dissemination of study results. This review provides a set of practical recommendations that can be used by the cancer research community when planning to involve or already involving patients in their clinical trial activities.

Long-term second primary cancer risk in adolescent and young adult (15-39 years) cancer survivors: a population-based study in the Netherlands between 1989 and 2018.

BACKGROUND: Few studies have comprehensively investigated the long-term second cancer risk among adolescent and young adult (AYA, aged 15-39 years) cancer survivors. This study investigated the long-term second cancer risk by including the full range of first and second cancer combinations with at least 10 observations in the Netherlands between 1989 and 2018. MATERIALS AND METHODS: First and second primary cancer data of all 6-month AYA cancer survivors were obtained from the nationwide population-based Netherlands Cancer Registry. Excess cancer risk compared to the general population was assessed with standardized incidence ratio (SIR) and absolute excess risk (AER) statistics up to 25 years after diagnosis. Cumulative incidences were estimated, using death as a competing risk factor. Analyses were carried out with and without applying multiple cancer rules. RESULTS: The cohort included 99 502 AYA cancer survivors. Male survivors had a 2-fold higher risk of developing any cancer compared to the general population, whereas this was around 1.3-fold in females. AERs were 17.5 and 10.1 per 10 000 person-years for males and females. The long-term excess risk of cancer was significantly higher for most first and second primary cancer combinations, but comparable and lower risk estimates were also observed. Application of the multiple cancer rules resulted in a noticeable risk underestimation in melanoma, testicular, and breast cancer survivors. Risk outcomes remained similar in most cases otherwise. The cumulative incidence of second cancer overall increased over time up to 8.9% in males and 10.3% in females at 25 years' follow-up. Highest long-term cumulative incidences were observed among lymphoma survivors (13.3% males and 18.9% females). CONCLUSIONS: AYA cancer survivors have a higher cancer risk compared to the general population for most cancers up to 25 years after their initial cancer diagnosis. Additional studies that investigate risk factors for the specific cancer type combinations are needed to develop personalized follow-up strategies.

The increasing burden of testicular seminomas and non-seminomas in adolescents and young adults (AYAs): incidence, treatment, disease-specific survival and mortality trends in the Netherlands between 1989 and 2019.

BACKGROUND: Testicular cancer incidence among adolescents and young adults (AYAs, aged 18-39 years at diagnosis) is increasing worldwide and most patients will survive the initial disease. Still, detailed epidemiological information about testicular cancer among AYAs is scarce. This study aimed to provide a detailed overview of testicular cancer trends in incidence, treatment, long-term relative survival and mortality by histological subtype among AYAs diagnosed in the Netherlands between 1989 and 2019. MATERIALS AND METHODS: Data of all malignant testicular cancers (ICD-code C62) were extracted from the Netherlands Cancer Registry. Mortality data were retrieved from Statistics Netherlands. European age-standardized incidence and mortality rates with average annual percentage change statistics and relative survival estimates up to 20 years of follow-up were calculated. RESULTS: A total of 12 528 testicular cancers were diagnosed between 1989 and 2019. Comparing 1989-1999 to 2010-2019, the incidence increased from 4.4 to 11.4 for seminomas and from 5.7 to 11.1 per 100 000 person-years for non-seminomas. Rising trends were most prominent for localized disease. Radiotherapy use in localized testicular seminomas declined from 78% in 1989-1993 to 5% in 2015-2019. Meanwhile, there was a slight increase in chemotherapy use. Most AYAs with localized seminomas and non-seminomas received active surveillance only (>80%). Overall, relative survival estimates remained well above 90% even at 20 years of follow-up for both seminomas and non-seminomas. Mortality rates declined from 0.5 to 0.4 per 100 000 person-years between 1989-1999 and 2010-2019. CONCLUSIONS: The incidence of seminoma and non-seminoma testicular cancers significantly increased in AYAs in the Netherlands between 1989 and 2019. There was a shift towards less-aggressive treatment regimens without negative survival effects. Relative survival estimates remained well above 90% at 20 years of follow-up in most cases. Testicular cancer mortality was already low, but has improved further over time, which makes survivorship care an important issue for these young adults.

Sexual quality of life of adolescents and young adult breast cancer survivors.

BACKGROUND: With increasing survival rates of adolescents and young adults (AYAs) with breast cancer, health-related quality of life (HRQoL) becomes more important. An important aspect of HRQoL is sexual QoL. This study examined long-term sexual QoL of AYA breast cancer survivors, compared sexual QoL scores with that of other AYA cancer survivors, and identified factors associated with long-term sexual QoL of AYA breast cancer survivors. MATERIALS AND METHODS: Data of the SURVAYA study were utilized for secondary analyses. Sexual QoL was assessed using the European Organization for Research and Treatment of Cancer Quality of Life cancer survivorship core questionnaire (EORTC QLQ-SURV100). Descriptive statistics were used to describe sexual QoL of AYA cancer survivors. Linear regression models were constructed to examine the effect of cancer type on sexual QoL and to identify factors associated with sexual QoL. RESULTS: Of the 4010 AYA cancer survivors, 944 had breast cancer. Mean sexual QoL scores of AYA breast cancer survivors ranged from 34.5 to 60.0 for functional domains and from 25.2 to 41.5 for symptom-orientated domains. AYA breast cancer survivors reported significantly lower sexual QoL compared to AYA survivors of other cancer types on all domains. Age, time since diagnosis, relationship status, educational level, chemotherapy, hormonal therapy, breast surgery, body image, and coping were associated with sexual QoL of AYA breast cancer survivors. CONCLUSIONS: AYA breast cancer survivors experience decreased sexual QoL in the long term (5-20 years) after diagnosis and worse score compared to AYA survivors of other cancer types, indicating a clear need to invest in supportive care interventions for those at risk, to enhance sexual well-being.

Health-related quality of life (HRQoL) after different axillary treatments in women with breast cancer: a 1-year longitudinal cohort study.

PURPOSE: As life expectancy continues to rise, post-treatment health-related quality of life (HRQoL) of breast cancer patients becomes increasingly important. This study examined the one-year longitudinal relation between axillary treatments and physical, psychosocial, and sexual wellbeing and arm symptoms. METHODS: Women diagnosed with breast cancer who received different axillary treatments being axilla preserving surgery (APS) with or without axillary radiotherapy or full axillary lymph node dissection (ALND) with or without axillary radiotherapy were included. HRQoL was assessed at baseline, 6- and 12-months postoperatively using the BREAST-Q and the European Organization for Research and Treatment of Cancer QoL Questionnaire Breast Cancer Module (EORTC QLQ-BR23). Mixed regression models were constructed to assess the impact of axillary treatment on HRQoL. HRQoL at baseline was compared to HRQoL at 6- and at 12-months postoperatively. RESULTS: In total, 552 patients were included in the mixed regressions models. Except for ALND with axillary radiotherapy, no significant differences in physical and psychosocial wellbeing were found. Physical wellbeing decreased significantly between baseline and 6- and 12-months postoperatively (p < 0.001, p = 0.035) and psychosocial wellbeing decreased significantly between baseline and 12 months postoperatively (p = 0.028) for ALND with axillary radiotherapy compared to APS alone. Arm symptoms increased significantly between baseline and 6 months and between baseline and 12 months postoperatively for APS with radiotherapy (12.71, 13.73) and for ALND with radiotherapy (13.93, 16.14), with the lowest increase in arm symptoms for ALND without radiotherapy (6.85, 7.66), compared to APS alone (p < 0.05). CONCLUSION: Physical and psychosocial wellbeing decreased significantly for ALND with radiotherapy compared to APS alone. Shared decision making and expectation management pre-treatment could be strengthened by discussing arm symptoms per axillary treatment with the patient.

Pregnancy-associated desmoid fibromatosis: A Dutch multi-centre retrospective study.

INTRODUCTION: The development of desmoid fibromatosis (DF) is associated with pregnancy. The current treatment consensus recommends active surveillance (AS). However, data in pregnancy-associated DF is scarce and it is uncertain whether AS is the best management strategy for this DF-subgroup. The aim of this study was to describe demographic, tumor, obstetric, treatment characteristics and treatment outcome in pregnancy-associated DF. METHODS: Female DF patients who were 18-50 years old at time of diagnosis (2000-2020) and had a history (≤5 years) of pregnancy at time of diagnosis were included. RESULTS: Overall, 62 patients were included. The most common locations were abdominal wall (74%), pelvis (10%) and extremities (10%). Mutational analysis was conducted in 31 patients of which 94% had CTNNB1-mutations. Ten patients (16%) were diagnosed during pregnancy, while the remainder were diagnosed after pregnancy with a median time from delivery to diagnosis of 19 months (1-60). The frontline management was AS in 38 patients (61%) of whom 12 (33%) developed progressive disease and surgery in 23 patients (37%). In total, 30 patients underwent surgery and five had local recurrence (17%). Positive resection margins were no prognostic factor. Nine patients received systemic treatment in second- or third-line. CONCLUSIONS: Pregnancy-associated DF generally has an indolent behavior, where our results underscore the difficulty of establishing a clear definition of this entity. This study shows that AS should be the frontline management strategy for pregnancy-associated DF. When active treatment is indicated, surgery is a good option with low recurrence rates, even with positive (R1) resection margins.

Setting the international research agenda for sarcoma together with patients and carers: first results of the Sarcoma Patient EuroNet (SPAEN) priority setting partnership.

BACKGROUND: Research in sarcomas has historically been the domain of scientists and clinicians attempting to understand the disease to develop effective treatments. This traditional approach of placing scientific rigor before the patient's reality is changing. This evolution is reflected in the growth of patient-centered organizations and patient advocacy groups that seek to meaningfully integrate patients into the research process. The aims of this study are to identify the unanswered questions regarding sarcomas (including gastrointestinal stromal tumors and desmoid fibromatosis) from patient, carer, and clinical perspectives and examine how patients and carers want to be involved in sarcoma research. METHODS: The Patient-Powered Research Network of Sarcoma Patients EuroNet set up a Priority Setting Partnership (PSP) in collaboration with stakeholders from the sarcoma research field. This PSP is largely based on the James Lind Alliance methodology. RESULTS: In total, 264 sarcoma patients (73%) and carers (27%) from all over the world participated in the online survey and covered the full spectrum of sarcomas. The topics mentioned were labeled in accordance with the Common Scientific Outline of the International Cancer Research Partnership and lists for potential research topics, advocacy topics, and requests for information were constructed. With regard to patient and carer involvement, 64% were very willing to be actively involved and mainly in the following areas: sharing perspectives, discussing patient-clinician interactions, and attending research meetings. CONCLUSIONS: The first results of this sarcoma PSP identified important research questions, but also important topics for patient advocacy groups and further improvement of information materials. Sarcoma patients and carers have a strong wish to be involved in multiple aspects of sarcoma research. The next phase will identify the top 10 research priorities per tumor type. These priorities will provide guidance for research that will achieve greatest value and impact.

Short- and long-term impact of cancer on employment and financial outcomes of adolescents and young adults (AYAs): a large population-based case-control registry study in the Netherlands.

BACKGROUND: Adolescent and young adult (AYA) cancer survivors, 18-39 years at initial cancer diagnosis, often self-report negative consequences of cancer (treatment) for their career. Less is known, however, about the objective impact of cancer on employment and financial outcomes. This study examines the employment and financial outcomes of AYA cancer survivors with nationwide population-based registry data and compares the outcomes of AYAs with cancer with an age- and sex-matched control population at year of diagnosis, 1 year later (short-term) and 5 years later (long-term). PATIENTS AND METHODS: A total of 2527 AYAs, diagnosed in 2013 with any invasive tumor type and who survived for 5 years, were identified from the Netherlands Cancer Registry (clinical and demographic data) and linked to Statistics Netherlands (demographic, employment and financial data). AYAs were matched 1 : 4 with a control population based on age and sex (10 108 controls). Analyses included descriptive statistics, chi-square tests, independent samples t-tests, McNemar tests and logistic regression. RESULTS: AYA cancer survivors were significantly less often employed compared with their controls 1 year (76.1% versus 79.5%, P < 0.001) and 5 years (79.3% versus 83.5%, P < 0.001) after diagnosis, and received more often disability benefits (9.9% versus 3.1% 1 year after diagnosis, P < 0.001; 11.2% versus 3.8% 5 years after diagnosis, P < 0.001). Unemployed AYAs were more often diagnosed with higher disease stages (P < 0.001), treated with chemotherapy (P < 0.001), radiotherapy (P < 0.001) or hormone therapy (P < 0.05) and less often with local surgery (P < 0.05) compared with employed AYAs 1 and 5 years after diagnosis. CONCLUSION: Based on objective, nationwide, population-based registry data, AYAs' employment and financial outcomes are significantly affected compared with age- and sex-matched controls, both short and long-term after cancer diagnosis. Providing support regarding employment and financial outcomes from diagnosis onwards may help AYAs finding their way (back) into society.

A qualitative study on the challenges health care professionals face when caring for adolescents and young adults with an uncertain and/or poor cancer prognosis.

BACKGROUND: Adolescents and young adults (AYAs, aged 18-39 years) with advanced cancer have an increased life expectancy due to improvements and refinements in cancer therapies, resulting in a growing group of AYAs living with an uncertain and/or poor cancer prognosis (UPCP). To date, no studies have examined the difficulties of health care professionals (HCPs) providing care to AYAs with a UPCP. This study aimed to understand the challenges in daily clinical practice experienced by HCPs from different disciplines who provide palliative as well as general care to AYAs with a UPCP. METHODS: HCPs from a variety of backgrounds (e.g. clinical nurse specialists, medical oncologists, neurologists psychologists) were invited for a semi-structured interview. The interviews were transcribed verbatim and analysed using reflexive thematic analysis. Two AYA patients were actively involved as research partners to increase the relevance of the study design and to optimise interpretation of results. RESULTS: Forty-nine HCPs were interviewed. Overall, we found that the threat of premature death within this young patient group increased emotional impact on HCPs and evoked a feeling of unfairness, which was an extra motivation for HCPs to provide the most optimal care possible. We generated four key themes: (i) emotional confrontation (e.g. feeling helplessness and experiencing a greater sense of empathy), (ii) questioning own professional attitude and skills, (iii) navigating uncertainty (e.g. discussing prognosis and end of life) and (iv) obstacles in the health care organisation (e.g. lack of knowledge and clarity about responsibilities). CONCLUSIONS: HCPs experienced unique emotional and practical challenges when providing care to AYAs with a UPCP. The results from this study highlight the need to develop an education module for HCPs treating AYAs with UPCP to increase their own well-being and optimise the delivery of person- and age-adjusted care.

Priorities and preferences of advanced soft tissue sarcoma patients starting palliative chemotherapy: baseline results from the HOLISTIC study.

INTRODUCTION: Palliative chemotherapy is the principal treatment of patients with advanced soft tissue sarcomas (STS); however prognosis is limited (median overall survival 12-19 months). In this setting, patient values and priorities are central to personalised treatment decisions. PATIENTS AND METHODS: The prospective HOLISTIC study was conducted in the UK and the Netherlands assessing health-related quality of life in STS patients receiving palliative chemotherapy. Participants completed a questionnaire before starting chemotherapy, including attitudes towards quality of life (QoL) versus length of life (LoL), decisional control preferences, and decisional conflict. Chi-square and Fisher's exact tests were used to evaluate associations between patient characteristics and preferences. RESULTS: One hundred and thirty-seven patients with advanced STS participated (UK: n = 72, the Netherlands: n = 65). Median age was 62 (27-79) years. Preference for extended LoL (n = 66, 48%) was slightly more common than preference for QoL (n = 56, 41%); 12 patients (9%) valued LoL and QoL equally (missing: n = 3). Younger patients (age <40 years) prioritised LoL, whereas two-thirds of older patients (aged ≥65 years) felt that QoL was equally or more important than LoL (P = 0.020). Decisional conflict was most common in patients who prioritised QoL (P = 0.024). Most patients preferred an active (n = 45, 33%) or collaborative (n = 59, 44%) role in treatment decisions. Gender, performance status, and country were significantly associated with preferred role. Concordance between preferred and actual role in chemotherapy decision was high (n = 104, 76%). CONCLUSIONS: Heterogeneous priorities and preferences among advanced STS patients support personalised decisions about palliative treatment. Considering individual differences during treatment discussions may enhance communication and optimise patient-centred care.

Beyond Teenage and Young Adult Cancer Care: Care Experiences of Patients Aged 25-39 Years Old in the UK National Health Service.

AIMS: Adolescents and young adults aged 15-39 years with cancer face unique medical, practical and psychosocial issues. In the UK, principal treatment centres and programmes have been designed to care for teenage and young adult patients aged 13-24 years in an age-appropriate manner. However, for young adults (YAs) aged 25-39 years with cancer, little access to age-specific support is available. The aim of this study was to examine this possible gap by qualitatively exploring YA care experiences, involving patients as research partners in the analysis to ensure robust results. MATERIALS AND METHODS: We conducted a phenomenological qualitative study with YAs diagnosed with any cancer type between ages 25 and 39 years old in the last 5 years. Participants took part in interviews or focus groups and data were analysed using inductive thematic analysis. Results were shaped in an iterative process with the initial coders and four YA patients who did not participate in the study to improve the rigor of the results. RESULTS: Sixty-five YAs with a range of tumour types participated. We identified seven themes and 13 subthemes. YAs found navigating the healthcare system difficult and commonly experienced prolonged diagnostic pathways. Participants felt under-informed about clinical details and the long-term implications of side-effects on daily life. YAs found online resources overwhelming but also a source of information and treatment support. Some patients regretted not discussing fertility before cancer treatment or felt uninformed or rushed when making fertility preservation decisions. A lack of age-tailored content or age-specific groups deterred YAs from accessing psychological support and rehabilitation services. CONCLUSIONS: YAs with cancer may miss some benefits provided to teenagers and young adults in age-tailored cancer services. Improving services for YAs in adult settings should focus on provision of age-specific information and access to existing relevant support.

The age-related impact of surviving sarcoma on health-related quality of life: data from the SURVSARC study.

BACKGROUND: Health-related quality of life (HRQoL) data of sarcoma survivors are scarce and the impact of age remains unclear. The aims of this population-based study were to (i) compare HRQoL scores amongst three age-groups [adolescents and young adults (AYA, aged 18-39 years), older adults (OA, aged 40-69 years) and elderly (aged ≥70 years)]; (ii) compare HRQoL of each sarcoma survivor age group with an age- and sex-matched normative population sample; (iii) determine factors associated with low HRQoL per age group. METHODS: Dutch sarcoma survivors, who were 2-10 years after diagnosis, were invited to complete the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30-questions questionnaire on HRQoL. RESULTS: In total, 1099 survivors (58% response rate) completed the questionnaire: 186 AYAs, 748 OAs and 165 elderly. The median time since diagnosis for all patients was 5.2 years. Bone sarcomas were seen in 41% of AYAs, 22% of OAs and in 16% of elderly survivors (P < 0.01). AYA and OA survivors reported statistically significant and clinically meaningful worse physical, role, cognitive, emotional and social functioning compared with a matched norm population, which was not the case for elderly survivors. AYAs reported significantly worse scores on emotional and cognitive functioning compared with OA and elderly survivors. Malignant peripheral nerve sheath tumour, osteosarcoma and chordoma were the subtypes of which survivors reported the lowest HRQoL scores in comparison with the norm. For all age groups, chemotherapy, having a bone sarcoma and having comorbidities were most frequently associated with low scores on HRQoL subscales, whereas a shorter time since diagnosis was not. CONCLUSION: In this nationwide sarcoma survivorship study, the disease and its treatment had relatively more impact on the HRQoL of AYA and OA survivors than on elderly survivors. These results emphasise the need for personalised follow-up care that not only includes risk-adjusted care related to disease relapse, but also age-adjusted care.

Diagnosed with a common cancer at an unusual age: causal attributions of survivors of adolescent and young adult colorectal cancer.

BACKGROUND: Insight into the causes of colorectal cancer (CRC) in adolescent and young adult (AYA) patients is limited. Without definitive information, patients often shape their own beliefs on the cause of their illness, developing causal attributions. This study aims to examine the causal attributions of CRC in AYA patients, compare these with middle-aged and older adults CRC patient groups and explore the association between causal attributions and psychological distress. METHODS: Two cross-sectional questionnaire studies were conducted among CRC survivors diagnosed between 1998 and 2007 using the population-based PROFILES registry. Three groups were defined by age at diagnosis: AYA (18-39 years; n = 67), middle-aged (40-70 years; n = 1993) and older adult survivors (70+ years; n = 1922). Causal attributions were assessed in a single free-text item from the Brief Illness Perception Questionnaire and psychological distress measured by the Hospital Anxiety and Depression Scale. RESULTS: AYA survivors most often attributed their CRC to heredity (36.4%), lifestyle (14.5%) and chance (10.9%). AYA survivors attributed their CRC to these causes more frequently than middle-aged and older adult CRC survivors. AYA survivors who attributed their CRC to causes out of their control (chance, heredity) showed significantly lower mean scores on anxiety (4.3 vs. 5.6; p < 0.01) compared to AYAs who reported causes within their control (lifestyle, stress). No significant difference was found for depression. CONCLUSION: AYA patients with CRC may benefit from in-depth discussion about the lack of known (biological) causes and how this does not directly imply a lifestyle or stress cause.

Ionising radiation exposure from medical imaging - A review of Patient's (un) awareness.

INTRODUCTION: Medical imaging is the main source of artificial radiation exposure. Evidence, however, suggests that patients are poorly informed about radiation exposure when attending diagnostic scans. This review provides an overview of published literature with a focus on nuclear medicine patients on the level of awareness of radiation exposure from diagnostic imaging. METHODS: A review of available literature on awareness, knowledge and perception of ionising radiation in medical imaging was conducted. Articles that met the inclusion criteria were subjected to critical appraisal using the Mixed Methods Appraisal Tool. RESULTS: 140 articles identified and screened for eligibility, 24 critically assessed and 4 studies included in synthesis. All studies demonstrated that patients were generally lacking awareness about radiation exposure and highlighted a lack of communication between healthcare professionals and patients with respect to radiation exposure. CONCLUSION: Studies demonstrate a need to better inform patients about their radiation exposure, and further studies focusing on nuclear medicine patients are particularly warranted. IMPLICATIONS FOR PRACTICE: Adequate and accurate information is crucial to ensure the principle of informed consent is present.

The effect of treatment on quality of life in patients with acromegaly: a prospective study.

OBJECTIVE: Acromegaly has a negative influence on health-related quality of life (HRQoL). Previous studies provide limited information on the course of HRQoL during treatment. This study aims to assess the effect of treatment on the course of HRQoL at six predefined time points. DESIGN: This prospective study examines HRQoL in treatment-naive patients before and during the first 2.5 years of acromegaly treatment. METHODS: Therapy-naive acromegaly patients completed three validated questionnaires (RAND-36, AcroQoL, and the Appearance Self-Esteem (ASE)) at six predetermined time points before, during, and after treatment. Outcomes were correlated to IGF1 levels and disease control status. RESULTS: Twenty-seven acromegaly patients completed the questionnaires at all time points. After treatment, all patients had controlled acromegaly. Scores of RAND-36 domains General health, Vitality and Health change, and all AcroQoL dimensions (except for Relations) improved during treatment (P ≤ 0.003); the largest changes were detected during the first year. Gender influenced HRQoL scores, since AcroQoL scores significantly improved in males but not in females. Over time, IGF1 levels were negatively correlated with HRQoL. After 2.5 years of follow-up, HRQoL of controlled patients was still lower than in the general population. CONCLUSION: HRQoL of acromegaly patients was considerably reduced at diagnosis. Disease control was associated with an improvement of HRQoL scores. Males showed a more pronounced improvement than females. The largest changes were detected in the first year of treatment. However, HRQoL during and after treatment remained impaired in acromegaly patients, emphasizing the need of additional support.

Embryonal and Alveolar Rhabdomyosarcoma in Adults: Real-Life Data From a Tertiary Sarcoma Centre.

AIMS: Embryonal and alveolar rhabdomyosarcoma (ERMS, ARMS) are subtypes of RMS that mainly occur in children, with relatively good outcomes. The incidence in adults is extremely low and survival is significantly worse compared with children. Data are scarce and literature generally combines all RMS subtypes, including pleomorphic RMS, which primarily occurs in adults and behaves more like undifferentiated pleomorphic sarcoma. The aim of this study was to evaluate patient and tumour characteristics, outcome and prognostic factors in adult patients with ERMS and ARMS. MATERIALS AND METHODS: All adult (18 years or older) ERMS and ARMS patients (presenting 1990-2016) were identified from a prospectively maintained database and were included in this analysis. RESULTS: Overall, 66 patients were included (42 men, 24 women). The median age at presentation was 28 years (range 18-71). The median overall survival for all ARMS (n = 42) and ERMS (n = 24) patients was 18 months, with a 5-year overall survival rate of 27%. Patients presenting with localised disease (n = 38, 58%) and metastatic disease (n = 25, 42%), had a 5-year overall survival rate of 36% and 11%, respectively. In univariate analysis we found alveolar subtype, fusion gene positivity, infiltrative tumour and metastatic presentation to be negative prognostic factors. CONCLUSION: Survival in adult ERMS and ARMS patients is poor and the current data may be useful in the design of trials with novel agents. Ideally, paediatric and adult oncologists should set up trials together to get a better understanding of biological, genetic and clinically relevant factors in this disease.

Health-related quality of life among cancer patients in their last year of life: results from the PROFILES registry.

PURPOSE: The aim of this study was to assess health-related quality of life (HRQoL) in the last year of life of cancer patients stratified by four periods of time before death. PATIENTS AND METHODS: Between 2008 and 2015, cancer patients were invited to participate in PROFILES (Patient Reported Outcomes Following Initial Treatment and Long-term Evaluation of Survivorship) registry studies. Patients were eligible for inclusion in this secondary analysis if they had been invited to complete the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) in their last year of life (N = 892). Four hundred fifty-eight patients (51%) responded. Descriptive statistics were used to describe the HRQoL of cancer patients in the last 3 months of life (N = 61), the last 3-6 months (N = 110), the last 6-9 months (N = 138), or the last 9-12 months of their life (N = 129). RESULTS: Patients in the last 3 months report a significant lower HRQoL, lower functioning, and higher symptom burden of fatigue and appetite loss compared to patients in different time periods before death (p < 0.008). Clinical relevance of the differences for global QoL, cognitive, and social functioning was large. Patients' HRQoL in the last year of life was significantly lower than that of the normative population (p < 0.001). CONCLUSIONS: All aspects of HRQoL are considerably impaired in patients with advanced cancer, with a marked lower HRQoL in the final months of life. This marked decline of HRQoL in the final months of life may be an indicator of approaching death and serve as an important trigger for end-of-life communication and decision-making about subsequent treatment and supportive care.

Posttraumatic growth and well-being among adolescents and young adults (AYAs) with cancer: a longitudinal study.

PURPOSE: This study examines posttraumatic growth (PTG) among adolescents and young adults (AYAs) with cancer, as well as its correlates and trajectories over time. The study also explores the buffering role of PTG on the associations between posttraumatic stress (PTS), health-related quality of life (HRQoL), and psychological distress. METHODS: A multicenter, longitudinal, prospective study was conducted among AYA cancer patients aged 14-39 years. One hundred sixty-nine patients completed a self-report measure of PTG (PTGI) and PTS (PDS) 6, 12, and 24 months after baseline (within the first 4 months of diagnosis). At 24-month follow-up, HRQoL (SF-36) and psychological distress (BSI-18) were also assessed. RESULTS: Among participants, 14% showed increasing PTG, 45% remained at a stable high PTG level, 14% showed decreasing PTG, and 27% remained at a stable low PTG level. AYAs who remained high on PTG were more often younger, female, and received chemotherapy. PTG level at 6-month follow-up was predictive of mental HRQoL (ß = 0.19; p = 0.026) and psychological distress (ß = -0.14; p = 0.043) at 24-month follow-up when corrected for PTS and sociodemographic and clinical covariates. No relationship between PTG and physical HRQoL was found. The interactive effects of PTS and PTG on outcomes were not significant, indicating that buffering did not take place. CONCLUSION: This study indicates that PTG is dynamic and predicts mental well-being outcomes but does not buffer the effects of PTS. Psychosocial interventions should focus on promoting PTG and reducing PTS in order to promote the adjustment of AYAs diagnosed with cancer.

Perceived impact of cancer among adolescents and young adults: Relationship with health-related quality of life and distress.

OBJECTIVE: To examine whether perceptions of the impact of cancer are related to health-related quality of life (HRQoL) and psychological distress among survivors of cancer in adolescence and young adulthood (AYA). METHODS: One hundred seventy-three AYA cancer survivors (aged 18-35 and 15-29 years at time of diagnosis) completed a mailed survey assessing impact of cancer (IOC-AYA), HRQoL (SF-36), and distress (BSI-18). Hierarchical linear regression models analyzed the independent effects of perceived impacts of cancer on HRQoL and distress after controlling for clinical and sociodemographic characteristics. Multivariate analyses also examined the extent to which positive and negative perceptions attenuated the effects of control variables on HRQoL and distress. RESULTS: Being unemployed or not in school, and self-reported health problems were significantly associated with worse physical HRQoL. Mental HRQoL and psychological distress appeared as a function of reporting both positive and negative impacts of cancer; mental health outcomes were better in AYAs reporting more positive and less negative impact of cancer in their lives. Perceived impact of cancer, in both positive and negative ways, attenuated the effects of sociodemographic and clinical factors on mental HRQoL and psychological distress. CONCLUSION: Results suggest that mental HRQoL and psychological distress, but not physical HRQoL, are a function of survivors' perceptions of how cancer has affected them and continues to affect them in both positive and negative ways. Findings suggest that opportunities for AYA cancer survivors to reframe or better understand the context of cancer in their lives may result in improved mental health outcomes.

Collaboration and Networking.

Awareness of the need for collaboration across pediatric and adult cancer to care for adolescents and young adults (AYAs) arose from the recognition of the unique characteristics of AYAs with cancer. Neither pediatric nor adult oncology hospital departments are able to provide age-appropriate care single handedly. The best way to bridge the gap in care of AYA cancer patients is to centralize aspects of their care within dedicated AYA care programs, including the following essential components: provision of developmentally appropriate and multidisciplinary (supportive) care, availability of AYA inpatient and outpatient facilities and healthcare professional AYA expertise as collaboration between adult and pediatric departments. Barriers are related to the slowly emerging evidence of benefit, cultural differences (collaboration between pediatric and adult oncology professionals), administrative and logistic challenges (small number of AYAs makes it difficult to create an AYA program in every hospital) and financial aspects (dependency on philanthropic funds). The sustainable development of an AYA program requires acceptance as a standard of care at the clinical and patient community and at government level. To improve the quality, equity and quantity of research and innovation in AYA cancer care across the world, it is necessary to join forces and collaborate in international networks to study issues such as the features of quality care, collaboration between pediatric and adult clinical teams, trial groups and professional societies, and AYA-specific groups such as Critical Mass, Canteen or European Network for Teenagers and Young Adults with Cancer.