Clinical trials in desmoid-type fibromatosis in children and adults:A systematic review

Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm, which occurs in children and adults, with a peak incidence in young adults. For the majority of the patients, DTF is a chronic and symptomatic disease, which affects health-related quality of life. Systemic treatment regimens tend to differ for patients treated by pediatric oncologists compared to medical oncologists. This systematic review identified 14 clinical trials in children and adults with DTF. Tumor response and progression-free survival rates varied widely between studies and study populations. Treatment choices for patients with DTF are based on a paucity of (randomized) trials. Treatment principles of DTF are similar in pediatric and adult oncology, but the treatment itself is different. This seems mostly driven by a lack of tyrosine kinase inhibitor (TKI) accessibility in pediatric oncology. An insufficient number of studies examined patient-reported outcomes, which are extremely important for patients with a chronic disease like DTF.</p

The views of cancer patients of Turkish, Moroccan, Surinamese, and Dutch-Caribbean descent on diagnosis, treatment and prognosis:A systematic literature review

Background: The number of international migrants is increasing worldwide. The four major non-western ethnic groups in the Netherlands are Turkish, Moroccan, Surinamese, and Dutch-Caribbean. This review examined the scientific literature on the views of cancer patients from these four ethnic groups on cancer diagnosis, treatment, and prognosis. Methods: A systematic literature review was conducted using the databases EMBASE, Medline Web of Science, and Cochrane Central Register. Studies with patients who were of Turkish, Moroccan, Surinamese, and Dutch-Caribbean descent were included. Both qualitative and quantitative studies were included, and thematic analysis was performed. The methodological quality was assessed using the Mixed Methods Appraisal Tool. Results: Thirteen studies were conducted in Turkey on Turkish cancer patients, while three were conducted in the Netherlands on Turkish and Moroccan cancer patients. Four themes emerged from the included studies: disclosure of diagnosis, communication, information provision, and decision-making. The majority of cancer patients in Turkey wanted information regarding their diagnosis and treatment. However, disclosure of a cancer diagnosis was rarely discussed with cancer patients in Turkey, whereas in the Netherlands it was provided directly. Family members in both the host and native countries had a strong influence on communication and decision-making. No literature on this topic for Surinamese or Dutch-Caribbean cancer patients was found. Conclusion: Although major ethnic groups live in host countries, there is a lack of knowledge on optimal communication and information disclosure on cancer to patients and their families. Policy summary: Further research into the views of ethnic groups on how to communicate about cancer is essential to ensuring that every patient receives optimal care and treatment.</p

Quality of life and experiences of sarcoma trajectories (the QUEST study):Protocol for an international observational cohort study on diagnostic pathways of sarcoma patients

Introduction Sarcomas are rare tumours with considerable heterogeneity. Early and accurate diagnosis is important to optimise patient outcomes in terms of local disease control, overall survival (OS) and health-related quality of life (HRQoL). Time to diagnosis is variable in bone as well as soft tissue sarcoma. Possible factors for a long time from first symptom to diagnosis (the total interval) include patient, tumour and healthcare characteristics, but until now the most relevant risk factors and its association with outcomes remain unknown. Our study aims to (1) quantify total interval, the time interval from first symptom until (histological) diagnosis; (2) identify factors associated with interval length and (3) determine the association between total interval and HRQoL, stage and tumour size at diagnosis, progression-free survival (PFS) and OS. Methods and analysis We will conduct a longitudinal, prospective, international, multicentre cohort study among patients aged ≥18 years with newly diagnosed bone or soft tissue sarcoma at eight centres (three in UK, five in The Netherlands). Patients will be asked to complete questionnaires at five points in time; one at diagnosis and at follow-up points of 3, 6, 12 and 24 months. Questionnaire data is collected within the Patient Reported Outcomes Following Initial treatment and Long term Evaluation of Survivorship (PROFILES) registry: an international data management system for collection of patient-reported outcomes. Clinical data will be extracted from patient records. The primary endpoint is HRQoL at diagnosis, measured with the EORTC QLQ-C30. Secondary endpoints are stage and tumour size at diagnosis, PFS, OS, additional patient-reported outcomes, such as quality-adjusted life years and psychological distress. Ethics and dissemination Ethical approval was given by the Health Research Authority and Research Ethics Committee for the United Kingdom (18/WA/0096) and medical ethical committee of Radboudumc for The Netherlands (2017-3881). Results will be presented in peer-reviewed journals and presented at meetings. Trial registration number NCT03441906

Same Journey, Different Paths:Caregiver Burden among Informal Caregivers of Adolescent and Young Adult Patients with an Uncertain or Poor Cancer Prognosis (UPCP)

A minority of adolescent and young adult cancer patients (AYA) live with an uncertain or poor prognosis (UPCP). Caring for a young, advanced cancer patient can lead to caregiver burden. This study aims to provide insight into burden on informal caregivers of AYA cancer patients with UPCP. In-depth, semistructured interviews were conducted with parents (n = 12), siblings (n = 7), friends (n = 7), and partners (n = 13). Thematic analysis was performed to derive themes from the data. Participants reported sleeping problems and stress. They struggle with uncertainty, fear, loss, and negative emotions. Family life is altered due to solely taking care of the children, but also the AYA. Contact with friends and family is changed. The relationship to the AYA can shift positively (e.g., becoming closer) or negatively (e.g., more conflict or no longer being attracted). Participants were under pressure, having to take on many responsibilities and multiple roles. In the financial domain, they report less income and often must continue working. A high amount of caregiver burden is experienced among informal caregivers of AYAs with UPCP. Yet only part of the impact appears to be age specific. Specific, age-adjusted interventions can be developed to lower the burden.</p

Same Journey, Different Paths:Caregiver Burden among Informal Caregivers of Adolescent and Young Adult Patients with an Uncertain or Poor Cancer Prognosis (UPCP)

A minority of adolescent and young adult cancer patients (AYA) live with an uncertain or poor prognosis (UPCP). Caring for a young, advanced cancer patient can lead to caregiver burden. This study aims to provide insight into burden on informal caregivers of AYA cancer patients with UPCP. In-depth, semistructured interviews were conducted with parents (n = 12), siblings (n = 7), friends (n = 7), and partners (n = 13). Thematic analysis was performed to derive themes from the data. Participants reported sleeping problems and stress. They struggle with uncertainty, fear, loss, and negative emotions. Family life is altered due to solely taking care of the children, but also the AYA. Contact with friends and family is changed. The relationship to the AYA can shift positively (e.g., becoming closer) or negatively (e.g., more conflict or no longer being attracted). Participants were under pressure, having to take on many responsibilities and multiple roles. In the financial domain, they report less income and often must continue working. A high amount of caregiver burden is experienced among informal caregivers of AYAs with UPCP. Yet only part of the impact appears to be age specific. Specific, age-adjusted interventions can be developed to lower the burden.</p

Sarcoma: Olaratumab - really a breakthrough for soft-tissue sarcomas?

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Adolescents and Young Adults Living With an Uncertain or Poor Cancer Prognosis:The "New" Lost Tribe

Historically, adolescent and young adult (AYA) patients with cancer, diagnosed for the first time at age 15 through 39 years, have often been identified as a "lost tribe" without a medical "home"; neither pediatric nor adult oncology services were able to provide ageappropriate care to this specific group. Internationally, AYA care programs are being established to bridge the gap between the age-defined healthcare worlds and to address the specific needs of AYAs with cancer. However, AYA care programs mostly focus on improving cure rates and addressing survivorship issues, and direct less attention to the unique needs of those living with an uncertain and/or poor cancer prognosis. Additionally, palliative care services are typically poorly equipped to address the age-specific needs of this group. Given that increasingly more AYAs with an uncertain and/or poor cancer prognosis are gaining life years because of novel treatments, and sometimes even face the prospect of longterm disease control, AYA care programs should address the unique palliative care needs of this "new" lost tribe within AYA oncology. This report provides a definition and description of the AYA population living with an uncertain and/or poor cancer prognosis in terms of epidemiologic, clinical, and psychosocial characteristics and challenges, and provides perspectives for future research and care initiatives. It also highlights the need to comprehensively examine the experience of AYAs who are living with uncertain and/or poor cancer prognosis to adjust best care practices for this unique group

The prolonged diagnostic pathway of young adults (Aged 25–39) with cancer in the United Kingdom:Results from the young adult cancer patient journey study

Purpose: Teenagers and young adults (TYAs; aged 13–24) experience prolonged intervals to cancer diagnosis. Insight into diagnostic intervals in young adults (YAs; aged 25–39) and sub-groups at risk for long intervals is lacking. We investigated the diagnostic pathway of YA cancer patients, examined patient and tumor characteristics associated with its length, and compared the patient interval length of our sample with a TYA cohort. Methods: In this cross-sectional survey YAs diagnosed with cancer in the UK in the past five years completed a questionnaire describing their patient (time from first symptom to first doctor consultation) and healthcare interval (from first consultation until consultation with a cancer specialist), sociodemographic, and clinical characteristics. Associations between characteristics and interval length were examined and compared with previously published data in TYAs. Results: Among 341 YAs the patient interval lasted ≥2 weeks, ≥1 month, and ≥3 months in 60%, 42%, and 21%, respectively, compared to 48%, 27%, and 12% in the TYA group. The healthcare interval lasted ≥2 weeks, ≥1 month, and ≥3 months in 62%, 40%, and 17% of YA patients, respectively. YAs with melanoma or cervical cancer were most likely to experience long intervals, whereas YAs with breast cancer and leukemia were most likely to experience short intervals. Conclusions: Most YAs were not seen by a cancer specialist within 2 weeks of GP consultation. Interval lengths in YAs were associated with cancer diagnosis. Patient intervals were longer among YAs than among TYAs. Our study highlights long diagnostic pathways among YAs and calls for more awareness among healthcare professionals about malignancies in this age group.</p

Patient-reported outcomes in randomized clinical trials of systemic therapy for advanced soft tissue sarcomas in adults:A systematic review

Background: This systematic review evaluates reporting of patient-reported outcomes (PROs) within randomized clinical trials (RCTs) for advanced soft tissue sarcoma (STS) patients. Methods: A systematic literature search from January 2000 – August 2022 was conducted for phase II/III RCTs evaluating systemic treatments in adult patients with advanced STS. Quality of PRO reporting was assessed using the CONSORT PRO extension. Results: Out of 7294 abstracts, 59 articles were included; comprising 43 RCTs. Only 15 RCTs (35%) included PROs, none as primary endpoints. Only 10 of these RCTs reported PROs, either in the primary (6/10) or secondary publication (1/10) or in both (3/10), with a median time interval of 23 months. The median CONSORT PRO adherence score was 5.5/14, with higher scores in publications focusing exclusively on PROs. Conclusion: These results highlight the need for improved and more consistent PRO reporting to inform patient care in the setting of advanced STS.</p

Therapeutic drug monitoring to personalize dosing of imatinib, sunitinib, and pazopanib:A mixed methods study on barriers and facilitators

Background:Personalized dosing based on measurement of individual drug levels and adjusting the dose accordingly can improve efficacy and decrease unnecessary toxicity of oncological treatment. For imatinib, sunitinib, and pazopanib, this therapeutic drug monitoring (TDM)-guided dosing is, however, not routinely used, despite accumulating evidence favoring individualized dosing. Therefore, we aimed to identify and quantify (potential) barriers and facilitators in TDM-guided dosing for imatinib, sunitinib, and pazopanib. Methods: We performed a mixed methods study among all stakeholders involved: patients, healthcare professionals (HCPs), pharmaceutical companies, and health insurance companies. During the first qualitative part of this study, we performed semi-structured individual interviews and one focus group interview to identify all (potential) barriers and facilitators, and during the second quantitative part of this study, we used a web-based survey to quantify these findings. The interviews addressed the six domains of the implementation of change model of Grol and Wensing: (1) the innovation itself; (2) the HCP; (3) the patient; (4) social context; (5) organizational context; and (6) finances, law, and governance. Results: In the qualitative study, we interviewed 20 patients, 18 HCPs and 10 representatives of pharmaceutical and health insurance companies and identified 72 barriers and 90 facilitators. In the quantitative study, the survey was responded by 66 HCPs and 58 patients. Important barriers were on the domain of the HCP, such as a lack of experience with TDM (36.4%), on the domain of the patient, such as lack of awareness of TDM (39.7%), and the processing time for measurement and interpretation of the TDM result (40.9%) (organizational domain). Important facilitators were education of HCPs (95.5%), education of patients (87.9%) and facilitating an overview of when and where TDM measurements are being performed (86.4%). Conclusion: We identified and quantified important barriers and facilitators for the implementation of TDM-guided dosing for imatinib, sunitinib, and pazopanib. Based on our results, the implementation strategy should mainly focus on educating both HCPs and patients and on the organizational aspect of TDM.</p