Search

PurposeThe role of chemotherapy (CT) and radiotherapy (RT) for management of extraskeletal osteosarcoma (ESOS) remains controversial. We examined disease outcomes for ESOS patients and investigated the association between CT/RT with recurrence and survival.Patients and methodsRetrospective review at 25 international sarcoma centers identified patients ≥18 years old treated for ESOS from 1971 to 2016. Patient/tumour characteristics, treatment, local/systemic recurrence, and survival data were collected. Kaplan-Meier survival and Cox proportional-hazards regression and cumulative incidence competing risks analysis were performed.Results370 patients with localized ESOS treated definitively with surgery presented with mainly deep tumours (n = 294, 80%). 122 patients underwent surgical resection alone, 96 (26%) also received CT, 70 (19%) RT and 82 (22%) both adjuvants. Five-year survival for patients with localized ESOS was 56% (95% CI 51%-62%). Almost half of patients (n = 173, 47%) developed recurrence: local 9% (35/370), distant 28% (102/370) or both 10% (36/370). Considering death as a competing event, there was no significant difference in cumulative incidence of local or systemic recurrence between patients who received CT, RT, both or neither (local p = 0.50, systemic p = 0.69). Multiple regression Cox analysis showed a significant association between RT and decreased local recurrence (HR 0.46 [95% CI 0.26-0.80], p = 0.01).ConclusionAlthough the use of RT significantly decreased local recurrences, CT did not decrease the risk of systemic recurrence, and neither CT, nor RT nor both were associated with improved survival in patients with localized ESOS. Our results do not support the use of CT; however, adjuvant RT demonstrates benefit in patients with locally resectable ESOS.

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]

Young adults (YAs), defined as individuals between the ages of 18 and 39 years, experience unique challenges when diagnosed with advanced cancer. Using the social constructivist grounded theory approach, we aimed to develop a theoretical understanding of how YAs live day to day with their diagnosis. A sample of 25 YAs (aged 22–39 years) with advanced cancer from across Canada participated in semi-structured interviews. Findings illustrate that the YAs described day-to-day life as an oscillating experience swinging between two opposing disease outcomes: (1) hoping for a cure and (2) facing the possibility of premature death. Oscillating between these potential outcomes was characterized as living in a liminal space wherein participants were unsure how to live from one day to the next. The participants oscillated at various rates, with different factors influencing the rate of oscillation, including inconsistent and poor messaging from their oncologists or treatment team, progression or regression of their cancer, and changes in their physical functioning and mental health. These findings provide a theoretical framework for designing interventions to help YAs adapt to their circumstance. [ABSTRACT FROM AUTHOR]

Adolescents and young adult (AYA) oncology care has evolved to address their unique supportive care needs, yet deficiencies remain. We planned a multiphase sequential mixed methods study beginning with focus groups (FGs) with AYAs receiving care at the Princess Margaret Cancer Centre, to improve supportive care delivery. Herewith, we report on the preliminary findings from first phase of project and outline future expected goals. Our findings suggest four major themes identified from FG discussion with AYAs: (1) having peer-driven support, (2) incorporating hybrid approach to information delivery, (3) integrating general practitioners in AYAs care, and (4) providing AYAs-specific supportive care programs. [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]

Adolescents and young adults (AYA) with cancer desire peer support and require support programs that address their unique needs. This study investigated the need for, and barriers to, peer support and preferences for digital peer navigation among AYA. A cross-sectional survey was administered to AYA, diagnosed with cancer between the ages of 15–39, at a cancer center and through social media. Descriptive summary statistics were calculated. Participants (n = 436) were on average 31.2 years (SD = 6.3), 3.3 years since-diagnosis (SD = 3.8), and 65% (n = 218) were women. Over three-quaters (n = 291, 76.6%) desired peer support from cancer peers, but 41.4% (n = 157) had not accessed peer support. Main access barriers were: Inconvenience of in-person support groups (n = 284, 76.1%), finding AYA with whom they could relate (n = 268, 72.4%), and finding AYA-specific support programs (n = 261, 70.4%). Eighty-two percent (n = 310) desired support from a peer navigator through a digital app, and 63% (n = 231) were interested in being a peer navigator. Participants indicated a greater need for emotional (n = 329, 90.1%) and informational support (n = 326, 89.1%) than companionship (n = 284, 78.0%) or practical support (n = 269, 73.6%) from a peer navigator. Foremost peer matching characteristics were cancer-type (n = 329, 88.4%), specific concerns (n = 317, 86.1%), and age-at-diagnosis (n = 316, 86.1%). A digital peer navigation program was desired by over 80% of a large Canadian sample of AYA and could potentially overcome the barriers AYA experience in accessing peer support. The design of a peer navigation program for AYA should consider the matching characteristics and multidimensional support needs of AYA. [ABSTRACT FROM AUTHOR]

Heat Shock Protein 90 (HSP90) is a potential drug target for cancer therapy as it is often dysregulated in several cancers, including lung, breast, pancreatic, and prostate cancers. In cancer, HSP90 fails to maintain the structural and functional integrity of its several client proteins which are involved in the hallmarks of cancer such as cell proliferation, invasion, migration, angiogenesis, and apoptosis. Several small molecule inhibitors of HSP90 have been shown to exhibit anticancer effects in vitro and in vivo animal models. However, a few of them are currently under clinical studies. The status and potential limitations of these inhibitors are discussed here. Studies demonstrate that several noncoding RNAs (ncRNAs) such as microRNAs (miRNAs) and long noncoding RNAs (lncRNAs) regulate HSP90 and its client proteins to modulate cellular processes to exhibit oncogenic or tumor suppressing properties. Over the last decade, miRNAs and lncRNAs have drawn significant interest from the scientific community as therapeutic agents or targets for clinical applications. Here, we discuss the detailed mechanistic regulation of HSP90 and its client proteins by ncRNAs. Moreover, we highlight the significance of these ncRNAs as potential therapeutic agents/targets, and the challenges associated with ncRNA-based therapies. This article aims to provide a holistic view on HSP90-regulating ncRNAs for the development of novel therapeutic strategies to combat cancer. • Heat Shock Protein 90 • Cancer therapy • Non-coding RNA • Therapeutic RNA [ABSTRACT FROM AUTHOR]

Background: The Children's Oncology Group clinical trial for intermediate risk rhabdomyosarcoma randomized participants to a combination of vincristine, dactinomycin, and cyclophosphamide (VAC) alone or VAC alternating with vincristine plus irinotecan (VAC/VI). Clinical outcomes were similar, but toxicity profiles differed. This study estimates the cost differences between arms from the health care system's perspective. Methods: A decision‐analytic model was used to estimate the incremental cost‐effectiveness ratio (ICER) of VAC versus VAC/VI. Protocol‐required or recommended medications and laboratory studies were included. Costs were obtained from national databases or supporting literature and inflated to 2019 US dollars. Demographic and outcome data were obtained from the clinical trial and directed chart reviews. Life‐years (LY) were estimated from life‐expectancy tables and discounted by 3% annually. Probabilistic sensitivity analyses and alternative clinical scenarios identified factors driving costs. Results: Mean direct medical costs of VAC and VAC/VI were $164,757 and $102,303, respectively. VAC was associated with an additional 0.97 LY and an ICER of $64,386/LY compared with VAC/VI. The ICER was sensitive to survival estimations and to alternative clinical scenarios including outpatient cyclophosphamide delivery (ICER $49,037/LY) or substitution of alternative hematopoietic growth factor schedules (ICER $73,191‐$91,579/LY). Applying drug prices from 2012 decreased the total costs of VAC by 20% and VAC/VI by 15% because of changes in dactinomycin and pegfilgrastim prices. Conclusions: Neither arm was clearly more cost‐effective. Pharmaceutical pricing and location of treatment drove costs and may inform future treatment decisions. Rising pharmaceutical costs added $30,000 per patient, a finding important for future drug‐pricing policy decisions. Lay Summary: Two chemotherapy regimens recently tested side‐by‐side for rhabdomyosarcoma had similar tumor outcomes, but different side effects.The health care costs of each regimen were compared; neither was clearly more cost‐effective.However, the costs of each treatment changed dramatically with choices of supportive medicines and location of treatment.Costs of treatment rose by 15% to 20% because of rising US drug costs not associated with the clinical trial. Vincristine, dactinomyin, and cyclosphosphamide alone, as well as alternating with vincristine plus irinotecan were not clearly more cost‐effective for intermediate‐risk rhadomyosarcoma. Pharmaceutical pricing and location of treatment drove costs and may influence future treatment decisions. [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]

Objective: Clinical experience suggests a high prevalence of emotional distress in patients with desmoid tumor (DT). We examine longitudinal Distress Assessment and Response Tool (DART) scores to estimate prevalence and persistence of distress, and compare cross-sectional data between DT and malignant sarcoma cohorts, to identify predictors of distress.Methods: Patients with DT completed DART at: T1-diagnosis, T2-during, T3-<6 months, and T4-≥6 months, post-treatment. DART includes patient-reported outcome measures of physical symptoms (ESAS-r), depression (PHQ-9), anxiety (GAD-7), and social difficulties (SDI-21). Descriptive prevalence and persistence of anxiety, depression, and wellbeing are reported, and mixed model regression analyses determine predictors of distress.Results: Between 2012 and 2018, a total of 152 DART screens from 94 patients with DT were completed (T1: n = 44, T2: n = 31, T3: n = 22, T4: n = 55). Patients had a mean age 40 years, 78% were female and DT locations were abdominal wall (48%), extremity (30%), and mesentery (22%). Moderate to severe ESAS-r scores (≥4) persisted at T4 for anxiety (20%), depression (13%), and poor wellbeing (31%). Compared to 402 patients with malignant sarcoma, patients with abdominal wall sited DT reported severe PHQ-9 and GAD-7 scores twice as frequently. Abdominal wall location, female sex, history of mood problems, and psychosocial concerns were significant predictors of anxiety, depression, and poor wellbeing in DT.Conclusions: Adults with DT experience persistently high emotional distress compared to patients with malignant sarcoma. Women with abdominal wall DT, prior mood, and current psychosocial concerns need early attention within multidisciplinary treatment settings to reduce persistent distress. [ABSTRACT FROM AUTHOR]

Objective: To describe fertility-related informational needs and practices, and to examine if demographic characteristics are related to these needs and practices.Methods: A needs assessment survey was conducted at three Canadian cancer centres.Results: 192 male cancer patients (Mage = 33.6) completed the survey. Most patients (70%) recalled having had a discussion with a health care provider regarding their fertility and 44% banked their sperm. Patients reported not getting all the information that they wanted, eg, the risk that a future child may have the same type of cancer (78%), and what was covered by insurance plans (71%). Barriers to sperm preservation were urgency to begin cancer treatment (49%), not planning to have a child in the future (47%) and worries that cancer could be passed on to future children (38%). Participants' age and being the parent of a child were significantly associated with having had a discussion about fertility. Participants' age, province, being the parent of a child and the desire for future children were significantly associated with fertility preservation.Conclusions: Discussions with health care providers were more frequent, and fertility preservation rates were higher than in past studies, but still not all patients' questions were answered. Misconceptions about passing on cancer to one's child, and that sperm preservation will delay treatment, should be dispelled. Health care providers can ask patients if they have any desire to have children in the future as a way to initiate a discussion of fertility preservation. Key information gaps and psychosocial resource needs are suggested to fully meet male cancer patients' fertility-related concerns. [ABSTRACT FROM AUTHOR]

Increasing accessibility of fertility preservation (FP) options has permitted women to retain fertility following anticancer therapies. Several published guidelines have made recommendations for FP however their implementation into practice is currently unknown. In this review, we aim to provide oncology clinicians practical information about FP options for post-pubescent female cancer patients and recommendations for care delivery in order to answer preliminary questions and help triage whether FP referral is appropriate. Herein, we present a resource for oncology providers to guide them with FP discussions. Key points that are discussed in this critical review include: i) All cancer patients beginning a new plan of care should be informed of potential fertility risk. ii) If a woman requests further information on FP interventions, referral to a FP clinic should be made. iii) Given the evolving technologies in this area, patients should be informed of those which are proven and unproven, with oocyte and embryo preservation recognized as standard practice. iv) Random start (independent of menstrual cycle day) techniques are available to minimize oncologic treatment delays. v) Specific protocols for ovarian stimulation may be center-specific. vi) There is unlikely an increased cancer recurrence risk as a result of stimulation protocols in women with hormone-sensitive cancers. vii) Lastly, given the absence of consensus in the literature, routine use of GnRH analogs is not recommended for all cancer patients, however may be considered in select cases, such as high-risk women in whom definitive FP is not possible or feasible. [ABSTRACT FROM AUTHOR]

Study Objective Alkylating agents are implicated in premature ovarian insufficiency. To optimize counseling regarding future ovarian function in survivors of adolescent cancer, we describe anti-Müllerian hormone (AMH) levels in female adolescents at diagnosis, during, and shortly after completion of chemotherapy. Design, Setting, Participants, Interventions, and Main Outcome Measures This was a prospective single-institution study. Participants were a mixed population of newly diagnosed postmenarchal female adolescents with malignancy. AMH was performed at diagnosis (T1), 6 months from diagnosis (T2), at end of therapy or 12 months [T3, whichever came first], 1 year after the end of therapy or 24 months from diagnosis [T4, whichever came first], and 18 months from the time of diagnosis (T5). All patients had baseline pelvic ultrasound examinations. Presence of menses and hot flashes were recorded at each time point. Results Sixteen participants with a median age at diagnosis of 14.3 years (range 12-17 years) were followed for 18.2 months (range, 14-24 months). Oncology diagnoses included leukemia, lymphoma, and sarcoma. Ten patients (62.5%) received alkylating agents with a median cumulative dose of 3041 mg/m 2 (range, 2639-6478 mg/m 2 ) of cyclophosphamide. Almost half (n = 7; 44%) experienced amenorrhea during treatment with resumption of menses in 6 of 7 patients (85%). Fifteen of 16 (94%) participants showed a decline in mean AMH levels by 6 months (T2) from diagnosis (15.8 IU/mL at T1 vs 6.5 IU/mL at T2; P = .003) and 12 of 15 (80%) showed at least some recovery of AMH (mean AMH at T4 = 13.2 IU/mL compared with 6.5 IU/mL at T2; P = .02). There was no difference in the mean decline nor recovery of AMH in those who did, vs did not receive cyclophosphamide. Conclusion To our knowledge, this is the largest series to date in adolescents showing that AMH is uniformly suppressed during cancer therapy and short-term recovery occurs in just more than half of the patients by 18-24 months. The contribution of short-term AMH measurements in predicting long-term ovarian function remains to be defined. Long-term follow-up with serial AMH levels is required to help predict those at risk for premature ovarian insufficiency. [ABSTRACT FROM AUTHOR]

Abstract: Background: Pancreatic tumors in children represent a very rare entity. We reviewed the clinical and pathologic features of pediatric patients with pancreatic tumors at a single institution. Methods: We conducted a retrospective review of cases diagnosed at the Hospital for Sick Children between 1986 and 2010. Results: Twenty-seven patients were diagnosed during the study period: 18 with solid pseudopapillary neoplasm (SPN), 6 with pancreatic endocrine neoplasia, and 3 with other tumors. Of the 27 children, 3 had associated syndromes, in specific tuberous sclerosis, von Hippel-Lindau, and polycystic ovarian syndrome. The most common symptoms were pain and vomiting in SPN and hypoglycemia and seizures in insulinomas. Magnetic resonance imaging and computed tomographic scan were equally accurate in determining size and site of origin of the tumor, and both were better than ultrasound. All patients underwent surgery (distal pancreatectomy in 14 cases, Whipple procedure in 8, other procedures in 5), which represented the only treatment for all but 3 cases. Seventeen patients (94%) with SPN are alive (median follow-up, 32 months), 3 of whom had positive margins. Two patients died: 1 male with SPN with malignant transformation and 1 with fibrosarcoma. Conclusions: We describe the largest single-institution study of pediatric pancreatic tumors. Females with SPN have an excellent outcome, even in presence of positive margins, suggesting that limited surgical resection may be appropriate for these patients. [Copyright &y& Elsevier]

By surveying men who are currently infertile (N = 251) and men who are potentially infertile (i.e., men with cancer; N = 195), the mental health consequences of reproductive masculinity, or the cultural assumption that men are virile and should be fathers, were investigated. There was no difference in depression levels between these two groups when controlling for demographic variables, suggesting that both groups of men have similar mental health needs. Since gendered notions of masculinity also suggest that men do not want to discuss their fertility health, their desire for online fertility-related social support was assessed. These findings suggest that most men do want to talk to others about fertility, which indicates that there is a need for more fertility-related social support. This research challenges some conceptions regarding masculinity, as men revealed an interest in accessing online social support related to fertility. [ABSTRACT FROM AUTHOR]