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The Children's Oncology Group defines intermediate-risk rhabdomyosarcoma as unresected FOXO1 fusion-negative disease arising at an unfavourable site or non-metastatic FOXO1 fusion-positive disease. Temsirolimus in combination with chemotherapy has shown promising activity in patients with relapsed or refractory rhabdomyosarcoma. We aimed to compare event-free survival in patients with intermediate-risk rhabdomyosarcoma treated with vincristine, actinomycin, and cyclophosphamide alternating with vincristine and irinotecan (VAC/VI) combined with temsirolimus followed by maintenance therapy versus VAC/VI alone with maintenance therapy. ARST1431 was a randomised, open-label, phase 3 trial conducted across 210 institutions in Australia, Canada, New Zealand, and the USA. Eligible patients were those aged 40 years or younger with non-metastatic FOXO1 -positive rhabdomyosarcoma or unresected FOXO1 -negative rhabdomyosarcoma disease from unfavourable sites. Two other groups of patients were also eligible: those who had FOXO1 -negative disease at a favourable site (excluding orbit) that was unresected; and those who were aged younger than 10 years with stage IV FOXO1 -negative disease with distant metastases. Eligible patients had to have a Lansky performance status score of 50 or higher if 16 years or younger and a Karnofsky performance status score of 50 or higher if older than 16 years; all patients were previously untreated. Patients were randomised (1:1) in blocks of four and stratified by histology, stage, and group. Patients received intravenous VAC/VI chemotherapy with a cyclophosphamide dose of 1·2 g/m2 per dose per cycle with or without a reducing dose of intravenous weekly temsirolimus starting at 15 mg/m2 or 0·5 mg/kg per dose for those who weighed less than 10 kg. The total duration of therapy was 42 weeks followed by 6 months of maintenance therapy with oral cyclophosphamide plus intravenous vinorelbine for all patients. Temsirolimus was withheld during radiotherapy and for 2 weeks before any major surgical procedure. The primary endpoint was 3-year event-free survival. Data were analysed with a revised intention-to-treat approach. The study is registered with ClinicalTrials.gov (NCT02567435) and is complete. Between May 23, 2016, and Jan 1, 2022, 325 patients were enrolled. In 297 evaluable patients (148 assigned to VAC/VI alone and 149 assigned to VAC/VI with temsirolimus), the median age was 6·3 years (IQR 3·0–11·3); 33 (11%) patients were aged 18 years or older; 179 (60%) of 297 were male. 113 (77%) of 148 patients were FOXO1 negative in the VAC/VI group, and 108 (73%) of 149 were FOXO1 negative in the VAC/VI with temsirolimus group. With a median follow-up of 3·6 years (IQR 2·8–4·5), 3-year event-free survival did not differ significantly between the two groups (64·8% [95% CI 55·5–74·1] in the VAC/VI group vs 66·8% [57·5–76·2] in the VAC/VI plus temsirolimus group (hazard ratio 0·86 [95% CI 0·58–1·26]; log-rank p=0·44). The most common grade 3–4 adverse events were anaemia (62 events in 60 [41%] of 148 patients in the VAC/VI group vs 89 events in 87 [58%] of 149 patients in the VAC/VI with temsirolimus group), lymphopenia (83 events in 65 [44%] vs 99 events in 71 [48%]), neutropenia (160 events in 99 [67%] vs 164 events in 105 [70%]), and leukopenia (121 events in 86 [58%] vs 132 events in 93 [62%]). There was one treatment-related death in the VAC/VI with temsirolimus group, categorised as not otherwise specified. Addition of temsirolimus to VAC/VI did not improve event-free survival in patients with intermediate-risk rhabdomyosarcoma defined by their FOXO1 translocation status and clinical factors. Novel biology-based strategies are needed to improve outcomes in this population. The Children's Oncology Group (supported by the US National Cancer Institute, US National Institutes of Health). [ABSTRACT FROM AUTHOR]

Adolescents and young adults (AYAs) with cancer, representing those between 15 and 39 years of age, face distinctive challenges balancing their life stage with the physical, emotional, and social impacts of a cancer diagnosis. These challenges include fertility concerns, disruptions to educational and occupational pursuits, issues related to body image and sexual health, and the need for age-appropriate psychosocial support within their communities. The Princess Margaret Cancer Centre (PM), a quaternary care center, established a specialized AYA program in 2014, offering holistic and developmentally tailored psychosocial support and currently, efforts are underway to expand this to other regions in the province to address the need for equitable access. The establishment process involves securing funding, conducting an environmental scan, identifying service gaps, developing clinical pathways, and implementing AYA supportive care. An accessible AYA program should also consider social determinants of health, social location, intersectionality, and an interdisciplinary health approach in understanding health inequities in AYA oncology care. This paper describes the processes implemented and challenges faced in creating a community-based AYA program beyond major resource-rich cities and efforts to address intersectionality. [ABSTRACT FROM AUTHOR]

Background: Adolescents and young adults (AYA, ages 15–39 years) affected by cancer have unique treatment, survivorship, and palliation concerns. Current oncology training does not focus on the distinctive needs of this demographic. Amid this recognition, the Canadian National AYA Cancer Task Force and Canadian Partnership Against Cancer have advocated the need for clinicians with formalized AYA experience. To address this need and standardize training, a national task force developed criteria for structured academic programs in AYA Oncology in Canada. Methods: Workshops were organized to identify and establish the fundamentals of practice in AYA Oncology through consensus. These workshops followed the pre‐existing rigorous process established by the Royal College of Physicians and Surgeons of Canada (Royal College) for new program development. The process includes: (i) developing the tasks associated with the discipline's practice, (ii) identifying the evidence trainees must provide to demonstrate tasks can be performed independently (the competence portfolio), (iii) developing training requirements and summarizing the knowledge, skills and attitudes required to perform these tasks, and (iv) identifying specific experiences essential to acquiring skills and demonstrating competent performance. Results: AYA Oncology is a recognized an Area of Focused Competence (AFC) by the Royal College. Conclusion: The AFC designation in AYA Oncology provides a standardized curriculum, training experience and accreditation process to attract oncologists, promote expertise and advance AYA oncology care. [ABSTRACT FROM AUTHOR]

Background: Due to ongoing disparity in the specialized care available to adolescents and young adults (AYAs) with cancer, this study aimed to understand the gaps and barriers to accessing care and preferences on types of solutions at a national Canadian level. Methods: A mixed-methods study involving an online survey and focus groups (FGs) was conducted among AYAs residing in different regions of Canada. Results: There were a total of 174 survey respondents, of whom the majority were between 30–39 years of age (n = 125, 71.8%). Of the 174 respondents, 36 (20.7%) participated in one of seven FGs. Triangulation of the results illustrated that AYAs are not appropriately informed about the long-term health risks of being treated for cancer and where/how to seek support. These results culminated into three themes: (1) the need for AYA relevant and timely information about health risks; by (a) producing health risk-related content with the AYA life stage in mind; (b) providing a guided "map" to help AYAs anticipate what they may experience, and (c) providing checklists to help AYAs navigate their experience; (2) need for tailored and timely supportive care including (a) establishing ongoing check-ins and (b) receiving navigation support, and (3) need for enhanced connections by creating (a) a space to gather, connect and seek mentorship and (b) a hub to access information. Conclusion: AYAs continue to lack sufficient support both during and following cancer and mechanisms are required to ensure longitudinal support is provided across jurisdictions and in all stages of the cancer journey. [ABSTRACT FROM AUTHOR]

Background: This study investigated prevalence of psychological distress, factors associated with distress, and experiences of Adolescents and Young Adults (AYAs) with cancer during the COVID‐19 pandemic. It also compared distress in this group to previously surveyed Canadian AYAs with cancer in 2018 by the Young Adults with Cancer in their Prime (YACPRIME) study. Methods: A cross‐sectional, online, self‐administered survey of AYAs diagnosed with cancer between 15 and 39 years of age was conducted. Psychological distress was measured by the Kessler Psychological Distress Scale (K10). Associations between variables and high psychological distress (K10 ≥ 25), and comparison of prevalence of psychological distress with the YACPRIME study were done using multivariable logistic regression. Summative qualitative content analysis analyzed participant experiences during this pandemic. Results: We included 805 participants. High psychological distress was present in over two‐thirds of the group (68.0%; 95% CI, 64.7%–71.2%). Employment impact during pandemic (AOR (adjusted odds ratio), 2.16; 95% CI, 1.41–3.31) and hematologic malignancy (AOR, 1.76; 95% CI 1.08–2.97) were associated with higher psychological distress, while older age [AOR, 0.95; 95% CI, 0.92–0.99] and personal income < $40,000 (AOR, 0.38; 95% CI, 0.24–0.58) were associated with lower distress. Adjusted odds of experiencing psychological distress among AYAs with cancer during pandemic compared to pre‐pandemic years was 1.85 (95% CI: 1.36–2.53). Overarching themes of pandemic experiences included: inferior quality of life, impairment of cancer care, COVID‐19 related concerns and extreme social isolation. Conclusion: AYAs diagnosed with cancer are experiencing high psychological distress during this pandemic. Distress screening and evidence‐based interventions to alleviate distress are essential. [ABSTRACT FROM AUTHOR]

Background: Adolescents and young adults (AYAs) diagnosed with cancer are at an increased risk of experiencing social isolation and loneliness secondary to their cancer and its treatment. The physical distancing measures implemented during the COVID-19 pandemic may have further increased loneliness among this group. This study examined the prevalence of loneliness and factors associated with loneliness among AYAs with cancer during this pandemic.Methods: We conducted a self-administered, online, cross-sectional survey of Canadian AYAs diagnosed with cancer between 15 and 39 between January and February 2021. Loneliness was measured using the 3-item UCLA Loneliness Scale. Factors associated with higher levels of loneliness were identified using multiple logistic regression.Results: The analysis included 805 AYAs. The prevalence of loneliness was 52.2% [N = 419, 95% CI (confidence interval) 48.7 to 55.6%]. Individuals who were 18-25 years old [AOR (adjusted odds ratio)1.60, CI 1.03-2.47, p = 0.035], currently undergoing cancer therapy (AOR 1.46, 95% CI 1.03-2.07, p = 0.035), who self-disclosed the presence of a pre-pandemic mental health condition (AOR 2.09, 95% CI = 1.22-3.58, p = 0.007), or were not in a relationship (AOR 2.22, 95% CI 1.57-3.14, p < 0.001) were more likely to report loneliness than others. Participants that lived in rural or remote locations were less likely to experience loneliness (AOR 0.59, 95%CI 0.40-0.87, p = 0.008).Conclusion: One in two AYAs with cancer are feeling lonely during the COVID-19 pandemic. Future studies for developing interventions to target loneliness, particularly for those at greater risk, are necessary to improve the health and quality of life of AYAs with cancer. [ABSTRACT FROM AUTHOR]

We aimed to describe the negative and positive impacts of changes in cancer care delivery due to COVID-19 pandemic for adolescents and young adults (AYAs) in Canada, as well as the correlates of negative impact and their perspectives on optimization of cancer care. We conducted an online, self-administered survey of AYAs with cancer living in Canada between January and February 2021. Multiple logistic regression was used to identify factors associated with a negative impact on cancer care. Of the 805 participants, 173 (21.5%) experienced a negative impact on their cancer care including delays in diagnostic tests (11.9%), cancer treatment (11.4%), and appointments (11.1%). A prior diagnosis of mental or chronic physical health condition, an annual income of <20,000 CAD, ongoing cancer treatment, and province of residence were independently associated with a negative cancer care impact (p-value < 0.05). The majority (n = 767, 95.2%) stated a positive impact of the changes to cancer care delivery, including the implementation of virtual healthcare visits (n = 601, 74.6%). Pandemic-related changes in cancer care delivery have unfavorably and favorably influenced AYAs with cancer. Interventions to support AYAs who are more vulnerable to the adverse effects of the pandemic, and the thoughtful integration of virtual care into cancer care delivery models is essential. [ABSTRACT FROM AUTHOR]

Purpose: Exercise and physical activity (hereafter, collectively referred to as PA) preferences and benefits are becoming increasingly well characterised in cancer survivors, yet evidence from adolescent and young adult cancer survivors (AYAs) is scant. We describe the overall PA behaviour and support preferences of AYAs and explore subgroup differences to inform AYA-specific research and support. Methods: AYAs diagnosed with cancer between the ages of 18–39 years, irrespective of current age, were approached in clinic at a large cancer centre for this cross-sectional survey that assessed self-reported demographics, medical history, PA behaviour and PA support preferences. Results: A total of 318 AYAs completed the survey. Approximately 40% of AYAs were not meeting PA guidelines, and only 5% reported engagement with cancer PA support services. Most AYAs wanted PA support (78%), to increase PA levels (70%), and were interested in engaging in PA interventions that were individually supervised (82%), home-based (79%), performed ≥ 3 days/week (75%) and for ≥ 30 minutes/session (78%), offered following treatment (63%), restricted by age (63%), and involved strength (83%), walking (78%), and flexibility (75%) exercise. PA preferences most often differed according to sex, treatment status, current PA behaviour and PA support setting. Conclusion: AYAs have a great capacity and interest in specific types of PA support. Poor engagement with existing PA support services highlights the need for further AYA-specific research. Better understanding of AYAs' unique PA behaviour and support preferences can inform the development of urgently needed research and support services for this understudied and rapidly growing population. [ABSTRACT FROM AUTHOR]

Purpose: Adolescents and young adults (AYA) with cancer are increasingly using the internet and social media (SM) for cancer-related information. AYA face specific challenges and thus require tailored resources that meet their needs. We describe the internet and SM preferences of AYA related to their cancer information seeking behaviors and their preferences for a future resource compared to middle-aged adults (MAA).Methods: Cancer patients completed a cross-sectional survey related to their internet and SM usage, cancer information, and preferences for future resources. Chi-square tests were used to compare AYA and MAA.Results: The mean (±SD) age in the AYA group was 30±6.1 years (n = 129); in MAA 55±6.7 years (n = 157). In general, AYA preferred internet sites over SM platforms for cancer-related information and prefer a website platform over a SM platform for a new resource. Few AYA were aware of hospital-based AYA-specific resources. MAA were less likely to use SM compared with AYA (13% vs 4%, p = 0.01); however, websites and SM platforms that were used were similar between the 2 groups. Participants endorsed having already researched certain topics - yet, these were also those desired in a new resource. Compared to MAA, AYA sought more information on diet/nutrition, physical activity, exercise/fitness, fertility, sexual health, and body image (all p values < 0.05).Conclusion: AYA and MAA use similar resources on the internet and SM, but AYA sought information related to specific needs. Development of future resources should focus on an internet-based platform rather than a SM platform, coupled with promoting awareness of the resource. [ABSTRACT FROM AUTHOR]

Purpose: Fertility is a concern for young women with breast cancer. We explore patient preferences for chemotherapy and whether women will trade-off survival benefits to maintain fertility following standardized information delivery. Methods: During a standardized interview, outcomes associated with adjuvant chemotherapy and 5 years of tamoxifen (CT) or 5 years of tamoxifen alone (NoCT) were described to participants. A threshold task was performed, in which each participant participated in two scenarios: (1) 10% absolute survival benefit from treatment and (2) 25% absolute survival benefit from treatment. The threshold point represented the reduction in fertility post-treatment that a participant would accept before she would trade-off CT benefit. Descriptive statistics were used to characterize participants. Demographic factors (age, marital status, parity at diagnosis, and education) associated with willingness to trade-off survival benefits were evaluated with logistic regression. Results: Analysis comprised 50 women with a median age of 34.5 years (range 25–39 years). Thirty-nine women (78%) completed university education. Thirty-four (68%) and 45 (90%) women in scenarios 1 and 2, respectively, were willing to trade-off all fertility (i.e., reduce fertility to 0% chance of conceiving naturally) to undertake CT and maintain survival benefits. Eight (16%) and three (6%) women in scenarios 1 and 2, respectively, chose to not pursue CT at all to maintain natural fertility. Regression analysis did not identify any variables that were predictive of participants' preferences. Conclusion: Most women with breast cancer are not willing to trade-off survival benefits of adjuvant therapy to maintain fertility. [ABSTRACT FROM AUTHOR]

1. Explain the impact of specialized palliative care on reducing high-intensity end-of-life care in adolescents and young adults with cancer 2. Identify subpopulations among adolescents and young adults with cancer who are at highest risk for reduced access to specialized palliative care A high proportion of adolescents and young adults (AYAs) with cancer (ages 15-39 years) receive high-intensity (HI) medical care at the end of life (EOL). We have previously shown that palliative care (PC) involvement in this population is associated with lower risk of HI-EOL care. Whether this association differs by specialized or general PC (SPC, GPC) is unknown. To evaluate the prevalence and predictors of SPC in AYAs with cancer and to evaluate the impact of SPC versus GPC on the intensity of EOL care for AYAs. A decedent cohort of AYAs with cancer who died between 2000 and 2017 in Ontario, Canada was assembled from registries and linked to population-based healthcare data. Based on prior studies, the primary composite measure HI-EOL care included any of intravenous chemotherapy <14 days from death, >1 ED visit, and >1 hospitalization or ICU admission <30 days from death. SPC and GPC were defined via previously validated algorithms based on physician billing codes (SPC if practice consisted of ≥50% PC-specific billing codes and GPC if PC-specific billing codes were between 10% and 50%). Of 7,122 AYAs, 2,140 (30%) received SPC and 942 (13.2%) received GPC. AYAs with hematologic malignancies, male AYAs, and rural AYAs were least likely to have access to SPC. No PC involvement compared to GPC was associated with higher odds of receiving HI-EOL care (OR 1.5; 95% CI, 1.3-1.8; P <.001). However, SPC was associated with the lowest risk of HI-EOL care (OR vs GPC 0.8; 95% CI, 0.7-0.9; P = 0.007). SPC was also associated with decreased odds of ICU admission compared with GPC (OR 0.7; 95% CI, 0.5-0.9; P = 0.006). SPC is associated with a lower risk of HI-EOL care in AYAs with cancer as compared to GPC. However, access to SPC remains a challenge. Our study supports the widespread provision of SPC to AYAs with cancer. [ABSTRACT FROM AUTHOR]

Context: Many adolescents and young adults (AYAs; 15-39 years) with cancer receive high intensity (HI) care at the end of life (EOL). Palliative care (PC) involvement in this population is associated with lower risk of HI-EOL care. Whether this association differs by specialized versus generalist PC (SPC, GPC) is unknown.Objectives: (1) To evaluate whether SPC had an impact on the intensity of EOL care received by AYAs with cancer; (2) to determine which subpopulations are at highest risk for reduced access to SPC.Methods: A decedent cohort of AYAs with cancer who died between 2000-2017 in Ontario, Canada was identified using registry and population-based data. The primary composite measure of HI-EOL care included any of: intravenous chemotherapy <14 days from death; more than one ED visit, more than one hospitalization or any ICU admission <30 days from death. Physician's billing codes were used to define SPC and GPC involvement.Results: Of 7,122 AYA decedents, 2,140 (30%) received SPC and 943 (13%) received GPC. AYAs who died in earlier years, those with hematologic malignancies, males and rural AYAs were least likely to receive SPC. No PC involvement was associated with higher odds of receiving HI-EOL care (odds ratio (OR) 1.5; p<0.0001). However, SPC was associated with the lowest risk of HI-EOL care (OR SPC versus GPC 0.8; p=0.007) and decreased odds of ICU admission (OR 0.7; p=0.006).Conclusion: SPC involvement was associated with the lowest risk of HI-EOL care in AYAs with cancer. However, access to SPC remains a challenge. [ABSTRACT FROM AUTHOR]

Adolescents and young adults (AYAs) represent an overlooked population in cancer survivorship care. Identifying the needs of AYAs can guide the development of tailored programs for this population. We conducted a cross-sectional descriptive analysis to identify biopsychosocial factors associated with AYA post-treatment supportive care needs and unmet needs using data obtained from the Experiences of Cancer Patients in Transitions Study of the Canadian Partnership Against Cancer, in collaboration with cancer agencies in the 10 Canadian provinces. The analysis focused on data from n = 530 AYAs between the ages of 18 and 34 who had undergone treatment within the past 5 years. Respondents reported a median of two moderate to big (MTB) physical needs (out of 9) and one unmet physical need, two MTB emotional needs (out of 6) with two unmet MTB emotional needs, and one (out of 5) practical need reported and one unmet MTB practical need. We found some common associations across supportive care domains. Income (lower) and more complex treatment were associated with high needs and unmet needs across the three domains. Respondents with a family doctor who was "very involved" in their cancer care had a lower number of unmet physical and emotional needs. Identifying those at risk of supportive care needs and developing tailored pathways in which they are proactively connected with tailored and appropriate resources and programs may help to reduce the number of unmet needs and improve cancer survivors' quality of life. [ABSTRACT FROM AUTHOR]

(1) Background: Adolescents and young adults (AYAs) who have been diagnosed with and treated for cancer have unique healthcare needs, but more research is needed to inform developmentally targeted cancer care for this population. The purpose of the current analyses was to describe the physical and psychosocial concerns and experiences of AYA cancer survivors during the post-treatment phase. (2) Methods: A national survey was conducted by the Canadian Partnership Against Cancer to evaluate the experiences and unmet needs of cancer survivors (≥18 years) within the first 5 years following cancer treatment. The current analyses were conducted on the AYA survivor population (18–34 years). (3) Results: A total of 575 surveys were completed by AYAs. Of these, 61% were female, 51% were married/partnered, and 52% were 1–3 years post treatment. Approximately three-quarters report their physical or emotional health as good/very good. Overall, 88% reported at least one physical concern [mean of 3.98+2.11 physical concerns (range 0–9)], 90% reported at least one emotional concern [mean of 3.77+1.75 emotional concern (range 0–6)], and 79% reported at least one practical challenge [mean of 2.39+1.28 practical concerns (range 0–5)]. The most common concerns were anxiety/worry about cancer returning (83%), fatigue/tiredness (78%), and depression/loss of interest in daily activities (66%). On average, 43% of those reporting a concern sought help. Common reasons for not seeking help included not wanting to ask, being told that it was normal to feel the way they did, or embarrassment. Of those who did seek help, 37% encountered difficulty obtaining assistance. (4) Conclusions: These results suggest that post-treatment AYA cancer survivors have a high rate and number of physical, psychosocial, and practical concerns and are often not seeking or receiving help to address these. Proactive approaches to characterizing and eliminating barriers to obtaining appropriate care are needed. [ABSTRACT FROM AUTHOR]

Background: Embryonal rhabdomyosarcoma of the cervix is a rare and aggressive malignancy that usually affects children and young adults.Case: We describe a 2-year-old patient who presented with a mass protruding through the vaginal introitus. Preoperative investigations including vaginoscopy, hysteroscopy, magnetic resonance imaging, and biopsies confirmed embryonal rhabdomyosarcoma, botryoid subtype, arising from the cervix. She was successfully treated with neoadjuvant chemotherapy and interval laparoscopic radical trachelectomy to achieve remission.Conclusion: Collaboration between the pediatric and adult surgical and medical oncology teams was critical to implement this fertility-sparing treatment strategy in such a young girl having this rare tumor. [ABSTRACT FROM AUTHOR]