Sarcoma Survivors Research Articles

Impact of Body Image Perception on Behavioral Outcomes in Chinese Adolescent and Young Adult Survivors of Sarcoma.

To identify the prevalence and predictive factors of body image dissatisfaction among Chinese adolescent and young adult (AYA) survivors of sarcoma and to evaluate its associations with behavioral outcomes. In total, 116 AYA survivors (response rate: 88%; 48.3% female; mean age 28.2 years, SD = 8.2 years) of osteosarcoma (49.1%) or soft-tissue sarcoma (50.9%) were recruited from an academic hospital. The survivors self-reported their perceived body image using the Body Image Scale. Behavioral outcomes were assessed using DSM-oriented scales of the ASEBA Adult Self-Report checklist. Multivariable linear regression was conducted to identify predictors of body image perception and to investigate the association between body image dissatisfaction and behavioral outcomes (adjusted for clinically relevant variables and corrected for false discovery rate). Mediation analyses were performed to examine the mediating effects of body image perception between clinical, treatment, or socioenvironmental risk factors and behavioral outcomes. At 15 years post-cancer diagnosis, one-third of the cohort (39.7%) reported dissatisfaction with their body image. The significant risk factors associated with body image dissatisfaction were being female (standardized coefficient estimate [Est] = 0.21, SE = 0.10; p = 0.047), surgery treatment (Est = 0.19, SE = 0.09; p = 0.046), and worse family functioning (Est = 0.27, SE = 0.10, p = 0.013). Body image dissatisfaction was associated with symptoms of depression (Est = 0.30, SE = 0.10; p = 0.005) and avoidant personality problems (Est = 0.37, SE = 0.11; p < 0.001). Negative body image perception significantly mediated the association between poor family functioning and avoidant personality problems (proportion-mediated 26.3%, p = 0.038). Body image dissatisfaction was associated with more significant internalizing symptoms of depression, anxiety, and avoidant personality. A negative body image perception may mediate the association between poor family functioning and psychological distress among adult survivors. The provision of psychosocial intervention early during the cancer care continuum may mitigate the negative impact of body image distress in Chinese AYA survivors.

Health-related quality of life, psychological issues and concerns among sarcoma survivors: a mixed method study.

We aimed to explore the health-related quality of life (HRQoL), psychological issues and concerns among sarcoma survivors in India and assess their satisfaction with nursing care. This study employed a sequential mixed-methods design, enrolling 100 sarcoma survivors from July to December 2021, with data collected using standardised questionnaires for HRQoL, depression, anxiety, stress, cognitive impairment and self-structured satisfaction with nursing care. Qualitative data were gathered through focused group discussions. The mean global health score among sarcoma survivors was 79.48 ± 16.26. A significant number of survivors had symptoms of mild-to-moderate depression (30%), severe anxiety (12%), stress (16%) and mild cognitive impairment (5%). Significant mean rank differences were observed between anxiety and financial difficulty (p < 0.05), emotional functioning (p < 0.001), cognitive functioning (p < 0.001), pain (<0.05), insomnia (p < 0.001), fatigue (p < 0.001), anorexia (p < 0.05) and nausea/vomiting (p < 0.001). Educational qualification had a significant association with depression and anxiety while family history of cancer emerged as a significant factor associated with anxiety and stress among survivors. Qualitative analysis revealed themes related to body image, societal discrimination, socio-economic impact, marriage concerns and fertility issues. Survivor satisfaction with nursing care was good. A substantial number of sarcoma survivors had an average HRQoL and experienced depression, anxiety and stress. Our study emphasizes the importance of holistic survivorship care, involving nurses in post-treatment support, and addressing societal discrimination and psychosocial concerns to enhance their quality of life. Our study calls for a holistic approach to sarcoma survivor care and emphasizes the importance of personalised survivorship care plans led by nurses to address the diverse needs of sarcoma survivors in India. Such plans should encompass strategies for managing depression, anxiety and stress, along with addressing body image concerns and social support.

Educational and occupational outcomes in Swedish children treated for sarcomas: A nationwide registry-based study.

Many children treated for cancer experience a negative impact on their academic performance; however, most studies of children treated for sarcomas have not investigated academic performance. Our aim was to explore how Swedish children treated for sarcomas perform academically, as well as how they adjust to life afterwards. We compared 167 pediatric sarcoma survivors with 776 matched, non-sibling controls without a history of cancer, in a retrospective cohort study using data from nationwide registries. Primary outcomes were grades at the end of compulsory education, high school eligibility, post-compulsory education (i.e., education after school Year 9), employment, and sickness or activity compensation. Pediatric sarcoma survivors were more likely to be ineligible for high school (odds ratio [OR] 1.76; p=.045) and more likely to fail Swedish (OR 2.12; p=.046), mathematics (OR 2.27; p=.011), and/or physical education (OR 2.24; p=.004), compared with controls. Survivors were less likely to have been employed (OR 0.58; p=.027) and received sickness or activity compensation more often (OR 2.49; p=.008) compared with controls. Pediatric sarcoma survivors have poorer academic performance compared to peers without cancer in multiple school subjects. Survivors seem to catch up during post-compulsory education, but might struggle to find employment.

A prospective study evaluating the association of patient-reported outcomes with activity level and sleep patterns in sarcoma survivors.

12085 Background: Sarcomas are a rare heterogeneous malignancy group affecting both sexes and all ages. Treatment is often intensive, resulting in long-term toxicities. Given the rarity of sarcoma, there is a paucity of data defining physical and emotional outcomes. The Cohort to Augment the Understanding of Sarcoma Survivorship Across the Lifespan (CAUSAL) was constructed at Vanderbilt University Medical Center (VUMC) to evaluate health-related outcomes. Methods: Sarcoma patients treated at VUMC from 2012 – present are enrolled into CAUSAL. Patient demographics, tumor and treatment data are abstracted from the electronic medical record. Participants complete surveys including PROMIS-57 consisting of seven domains including physical function, sleep disturbance, fatigue, and satisfaction with participation in social roles, which are reported here. Participants who have completed treatment are given a FitBit to track activity level and sleep over 12 weeks. Data are synced to an online platform, Fitabase. Body mass index (BMI) is assessed at study entry. For each participant, FitBit data were summarized using medians. To estimate the association between PROMIS-57 scores and activity and sleep data (FitBit), linear regression models were fit controlling for age, sex, and BMI of participants. Results: Of 306 CAUSAL participants who completed the PROMIS-57, 193 were post completion of sarcoma therapy. Of these, 76 also had FitBit data and are included in this analysis. Median step count was 5826 (quartiles: 3668, 7967), which was lower than average Fit Bit step count data for the US population (8170). Median daily active minutes was 252 minutes (quartiles: 174, 307). Median duration of sleep was 447 minutes (quartiles:381, 474) which was greater than the average reported by FitBit for the general US population (436 min). Step count was correlated with higher physical function score (p &lt; 0.001). A 10-point increase in physical function led to an average increase of 1291 (95% CI: 609,1973) steps when adjusting for age and sex. Compared with participants receiving treatment within 30 days prior to CAUSAL enrollment, participants not receiving treatment had 4.50 (95% CI: 1.53, 7.47) higher physical function score and 6.26 (95% CI: 1.87, 10.65) higher satisfaction in their social roles. There was no correlation between minutes of sleep recorded by FitBit and PROMIS responses. Conclusions: Using PROMIS-57, and FitBits, we demonstrated an association between higher step counts and self-reported higher physical function but did not find a correlation between FitBit recorded activity level/sleep and self-reported sleep disturbance or fatigue scores. Further, our data suggests patients with sarcoma sleep more and walk less than the general US population. As enrollment continues in CAUSAL, we will focus on PROMIS scores over time and their association with activity level and sleep.

Cumulative burden of late, major surgical intervention in survivors of childhood cancer: a report from the Childhood Cancer Survivor Study (CCSS) cohort.

Multimodal cancer therapy places childhood cancer survivors at increased risk for chronic health conditions, subsequent malignancies, and premature mortality as they age. We aimed to estimate the cumulative burden of late (>5 years from cancer diagnosis), major surgical interventions among childhood cancer survivors, compared with their siblings, and to examine associations between specific childhood cancer treatments and the burden of late surgical interventions. We analysed data from the Childhood Cancer Survivor Study (CCSS), a retrospective cohort study with longitudinal prospective follow-up of 5-year survivors of childhood cancer (diagnosed before age 21 years) treated at 31 institutions in the USA, with a comparison group of nearest-age siblings of survivors selected by simple random sampling. The primary outcome was any self-reported late, major surgical intervention (defined as any anaesthesia-requiring operation) occurring 5 years or more after the primary cancer diagnosis. The cumulative burden was assessed with mean cumulative counts (MCC) of late, major surgical interventions. Piecewise exponential regression models with calculation of adjusted rate ratios (RRs) evaluated associations between treatment exposures and late, major surgical interventions. Between Jan 1, 1970, and Dec 31, 1999, 25 656 survivors were diagnosed (13 721 male, 11 935 female; median follow-up 21·8 years [IQR 16·5-28·4]; median age at diagnosis 6·1 years [3·0-12·4]); 5045 nearest-age siblings were also included as a comparison group. Survivors underwent 28 202 late, major surgical interventions and siblings underwent 4110 late, major surgical interventions. The 35-year MCC of a late, major surgical intervention was 206·7 per 100 survivors (95% CI 202·7-210·8) and 128·9 per 100 siblings (123·0-134·7). The likelihood of a late, major surgical intervention was higher in survivors versus siblings (adjusted RR 1·8, 95% CI 1·7-1·9) and in female versus male survivors (1·4; 1·4-1·5). Survivors diagnosed in the 1990s (adjusted RR 1·4, 95% CI 1·3-1·5) had an increased likelihood of late surgery compared with those diagnosed in the 1970s. Survivors received late interventions more frequently than siblings in most anatomical regions or organ systems, including CNS (adjusted RR 16·9, 95% CI 9·4-30·4), endocrine (6·7, 5·2-8·7), cardiovascular (6·6, 5·2-8·3), respiratory (5·3, 3·4-8·2), spine (2·4, 1·8-3·2), breast (2·1, 1·7-2·6), renal or urinary (2·0, 1·5-2·6), musculoskeletal (1·5, 1·4-1·7), gastrointestinal (1·4, 1·3-1·6), and head and neck (1·2, 1·1-1·4) interventions. Survivors of Hodgkin lymphoma (35-year MCC 333·3 [95% CI 320·1-346·6] per 100 survivors), Ewing sarcoma (322·9 [294·5-351·3] per 100 survivors), and osteosarcoma (269·6 [250·1-289·2] per 100 survivors) had the highest cumulative burdens of late, major surgical interventions. Locoregional surgery or radiotherapy cancer treatment were associated with undergoing late surgical intervention in the same body region or organ system. Childhood cancer survivors have a significant burden of late, major surgical interventions, a late effect that has previously been poorly quantified. Survivors would benefit from regular health-care evaluations aiming to anticipate impending surgical issues and to intervene early in the disease course when feasible. US National Institutes of Health, US National Cancer Institute, American Lebanese Syrian Associated Charities, and St Jude Children's Research Hospital.

Psychological Well-Being, Self-Esteem, Quality of Life and Gender Differences as Determinants of Post-Traumatic Growth in Long-Term Knee Rotationplasty Survivors: A Cohort Study.

Rotationplasty (RP) is a special surgical technique for bone tumors of the lower limb and is the chosen procedure for children under 6 with bone sarcoma in the distal femur. Leg reconstruction results in an unusual aspect of the limb potentially giving life-long emotional outcomes, especially considering the young age of most RP patients. Although the high level of the quality of life of these patients has been previously reported, aspects related to long-term psychological well-being, self-esteem and life satisfaction, particularly regarding the gender, procreation and parenting, have never been explored. The aim of this study was to assess the general degree of psychological well-being of RP patients, with specific reference to gender, procreation and parenting. Twenty long-term RP survivors of high-grade bone sarcoma participated in the study. They were administered the following validated questionnaires: HADS for psychological well-being (degree of anxiety and depression), Temperament and Character Inventory (TCI), RSES for self-esteem, SF-36 for quality of life, SWLS extended to life satisfaction, and ABIS for body image integration. Data on education, marriage, employment and parenthood were gathered. All the scores obtained were very close to normal references. The only gender difference was found for the TCI Cooperativeness scale, which was higher in women than in men. A satisfactory psychological well-being in terms of both self-esteem and integration of the prosthetic joint limb into one's body image, with relatively limited amount of anxiety/depression, good quality of life, and good temperament and character traits, was found. No major gender differences were reported.

Abstract 5759: Accelerated cardiac aging in adolescent and young adult cancer survivors previously treated with cardiotoxic therapy

Abstract Cancer in adolescents and young adults (AYA) is defined as cancer occurring between the ages of 15-39 years. Although cancer is the most common disease-related cause of death in this age group world-wide, the highly favorable overall 5-year survival rate for AYA cancer patients is creating a large and growing segment of the cancer survivorship population. Young cancer survivors have rates of comorbidities, frailty, and other phenotypes that are in line with that of individuals decades older, suggesting that cancer and/or treatment exposures accelerate the aging process in these survivors. Cardiotoxicity due to anthracycline and/or radiation exposure is a major contributor to poor cardiovascular health in AYA cancer survivors, yet the presence and the potential impact of accelerated cardiac aging remains unclear. We hypothesized that hearts in AYA cancer patients exposed to cardiotoxic treatment modalities have a functional phenotype that mirrors heart function in non-treatment exposed individuals who are decades older. In this cross-sectional study, 424 echocardiograms from 127 AYA Hodgkin lymphoma (N=98) and sarcoma (N=29) survivors who were treated with anthracyclines and/or radiation were reviewed for eight functional measures (left ventricular ejection fraction, A-wave, E-wave, E/A ratio, E’-wave, posterior wall thickness, mitral deceleration time, and left ventricular end-diastolic dimension). The median anthracycline dose received for the population was 291.0 mg/m2 (SD: 133.7) and 44.9% were exposed to radiation to the chest. At last follow-up, 8.3% were taking cardiac medications and 15.8% had been diagnosed with a cardiovascular disease during an average follow-up period of 5.6 years. Echocardiograms were obtained on average 1.3 years post-diagnosis (range: 0-14) and were grouped based at AYA survivor age at procedure (20-29 years, 30-39 years, and 40-49 years). Left ventricular ejection fraction (LVEF) and E’-wave were significantly lower than controls in all three age groups (P&amp;lt;0.001), with four other measures being significant in both of the two younger age groups (P&amp;lt;0.05). Strikingly, 90% of the population had more than one functional measure that was indicative of hearts at least two decades older than the patient at time of echocardiogram with AYA survivors having an average of 4.2 measures displaying evidence of accelerated cardiac aging. These results suggest that AYA cancer survivors exposed to cardiotoxic treatment modalities have a high burden of poor cardiac function and that accelerated cardiac aging may responsible. Citation Format: Michelle A. T. Hildebrandt, Cole J. Bernstein, Sairah Ahmed, J. Andrew Livingston, Efstratios Koutroumpakis, Michael E. Roth, Jose Banchs. Accelerated cardiac aging in adolescent and young adult cancer survivors previously treated with cardiotoxic therapy. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 5759.

Early postoperative gain in activity levels of lower extremity sarcoma survivors positively affects long-term physical activity and performance

PurposeLittle is known about the effect of sports activity levels on health-related quality of life (HRQOL) in long-term survivors of lower-extremity sarcoma.MethodsEighty-three long-term survivors of bone and soft tissue sarcoma of the lower extremities with a median follow-up of 14 (range: 5–35) years completed the University of California and Los Angeles (UCLA) activity scores before tumor resection, 1 year after surgery and at the latest follow-up, as well as a Short Form 36 (SF-36) health survey at the latest follow-up. Simple linear regression models as well as stepwise variable selection with Akaike information criterion (AIC) were undertaken.ResultsThe preoperative UCLA activity level (median: 9, range: 2–10) dropped to a median of 4 (range: 1–10) 1 year after surgery before increasing to a score of 6 (range: 2–10) 5 years after surgery. The long-term SF-36 physical health component summary score (PCS) was 49 (SD: 9), and the mental health component summary score (MCS) was 54 (SD: 7). A linear model with stepwise variable selection identified a negative correlation of PCS with age at surgery (estimate: –0.2; p = 0.02), UCLA score at the last follow-up (estimate: 1.4; p = 0.02) and UCLA score 1 year after surgery (estimate: 1.0; p = 0.02).ConclusionAs not only the final activity levels but also the status immediately after surgery affect the PCS, higher early activity levels should be a goal of modern rehabilitation after sarcoma treatment. Further studies are needed to weigh the potential postoperative risks of higher sport activity levels against the benefits described in this study.Level of evidence: Level 4.

Impaired social functioning in adolescent and young adult sarcoma survivors: Prevalence and risk factors.

Sarcomas account for almost 11% of all cancers in adolescents and young adults (AYAs; 18-39years). AYAs are increasingly recognized as a distinct oncological age group with its own psychosocial challenges and biological characteristics. Social functioning has been shown to be one of the most severely affected domains of health-related quality of life in AYA cancer survivors. This study aims to identify AYA sarcoma survivors with impaired social functioning (ISF) and determine clinical and psychosocial factors associated with ISF. AYAs from the population-based cross-sectional sarcoma survivorship study (SURVSARC) were included (n=176). ISF was determined according to the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 social functioning scale, and age- and sex-matched norm data were used as reference. The median time since diagnosis was 6.2years (range, 1.8-11.2). More than one-quarter (28%) of AYA sarcoma survivors experienced ISF. Older age, higher tumor stage, comorbidities, lower experienced social support, uncertainty in relationships, feeling less attractive, sexual inactivity, unemployment, and financial difficulties were associated with ISF. In a multivariable analysis, unemployment (OR, 3.719; 95% CI, 1.261-10.967) and having to make lifestyle changes because of financial problems caused by one's physical condition or medical treatment (OR, 3.394; 95% CI, 1.118-10.300) were associated with ISF; better experienced social support was associated with non-ISF (OR, 0.739; 95% CI, 0.570-0.957). More than one-quarter of AYA sarcoma survivors experience ISF long after diagnosis. These results emphasize the importance of follow-up care that is not only disease-oriented but also focuses on the psychological and social domains. Sarcomas account for almost 11% of all cancers in adolescents and young adults (AYAs; 18-39years). The AYA group is increasingly recognized as a distinct oncological age group with its own psychosocial challenges and biological characteristics. Social functioning has been shown to be severely affected in AYA cancer survivors. A population-based questionnaire study to identify AYA sarcoma survivors with impaired social functioning (ISF) and determine factors associated with ISF was conducted. More than one-quarter of AYA sarcoma survivors experience ISF long after diagnosis. These results emphasize the importance of follow-up care that is not only disease-orientated but also focuses on the psychological and social domains.

Associations between Lifestyle Factors and Neurocognitive Impairment among Chinese Adolescent and Young Adult (AYA) Survivors of Sarcoma.

The effect of lifestyle on neurocognitive impairment among cancer survivors remain an understudied area. This study explored the association between lifestyle factors and neurocognitive outcomes (specifically, attention, memory, processing speed and cognitive flexibility) in AYA survivors (aged 15-39 years) of sarcoma. This study recruited 116 AYA survivors (age 28.2 (SD = 8.2) years), who were diagnosed with osteosarcoma (49%) or soft-tissue sarcoma (51%) at age 13.3 (SD = 7.2) years. The neurocognitive battery included measures of attention, memory, motor-processing speed, and cognitive flexibility. Survivors reported health-damaging practices, which included: physical inactivity, smoking, alcohol intake, inadequate sleep (<7 h of actual sleep/day), sleep-related fatigue (Multidimensional Fatigue Scale) and long working hours (>9 h/day). General linear modeling was conducted to examine the association between lifestyle factors and neurocognitive outcomes, adjusting for age at diagnosis, sex, education attainment and clinical/treatment variables. At 14.9 (SD = 7.6) years post-diagnosis, survivors demonstrated impairment in attentiveness (4.3-13.0%), processing speed (34.5%) and cognitive flexibility (18.1%). Nearly half (45.7%) had developed a chronic health condition (CHC). Low physical activity (estimate = -0.97, p = 0.003) and sleep-related fatigue (estimate = -0.08, p = 0.005) were associated with inattention. Survivors who worked >9 h/day (n = 15) demonstrated worse attention (estimate = 5.42, p = 0.023) and cognitive flexibility (estimate = 5.22, p = 0.005) than survivors who worked ≤9 h/day (n = 66). Interaction analysis (CHCs*physical activity) showed that survivors who developed CHCs and reported low physical activity had worse attention (p = 0.032) and cognitive-flexibility (p = 0.019) scores than other subgroups. Treatment-related CHCs, coupled with continued physical inactivity, may exacerbate inattention and executive dysfunction among survivors. Long working hours and sleep-related fatigue are associated with worse functioning; this finding should be validated with prospective assessment of work-related stressors and objective sleep measures.

Quantifying muscle strength, size, and neuromuscular activation in adolescent and young adult survivors of musculoskeletal sarcoma: Identifying correlates and responses to functional strengthening

BackgroundMedical and surgical treatment for musculoskeletal sarcoma (MSS) place survivors at risk for impairments in muscle properties including muscle strength, muscle size, and neuromuscular activation. The purpose of this study was to explore muscle properties, gross motor performance, and quality of life (QoL) and the changes in response to a 6-week functional strengthening intervention (PT-STRONG) in MSS survivors of childhood cancer (CCS). MethodsEight lower extremity MSS CCS (13–23 years old) performed baseline testing and three completed PT-STRONG. Participants completed measurements of knee extension strength using handheld dynamometry, vastus lateralis (VL) and rectus femoris (RF) muscle thickness using ultrasonography at rest, and neuromuscular activation using electromyography during strength testing and a step-up task. Participants also completed gross motor and QoL assessments. ResultsCompared with the non-surgical limb, MSS CCS had lower surgical limb knee extension strength, VL muscle thickness, and RF step-up muscle rate of activation (RoA). Compared with normative values, MSS CCS had decreased bilateral knee extension strength, gross motor performance, and physical QoL. Positive correlations among muscle strength, muscle thickness, and gross motor performance were identified. After PT-STRONG, MSS CCS had improvements in VL muscle thickness, VL and RF RoA duing step-up, gross motor performance, and physical QoL. ConclusionsPositive association between larger muscle thickness with greater knee extension strength, and higher knee extension strength with better gross motor performance indicate that comprehensive physical therapy assessment and interventions that identify and target impairments in muscle properties to guide clinical decision making should be considered for MSS CCS into survivorship.

Fear of Cancer Recurrence in Sarcoma Survivors: Results from the SURVSARC Study.

A cross-sectional study was conducted among sarcoma survivors 2 to 10 years after diagnosis. Patients completed the Cancer Worry Scale (CWS), the global health status subscale of the EORTC QLQ-C30 and a custom-made questionnaire on follow-up care. In total, 1047 patients were included (response rate 55%). The prevalence of high FCR was 45%. Factors associated with high FCR were female sex with 1.6 higher odds (95% CI 1.22-2.25; p = 0.001); having ≥1 comorbidities and receiving any treatment other than surgery alone with 1.5 (95% CI 1.07-2.05; p = 0.017) and 1.4 (95% CI 1.06-1.98; p = 0.020) higher odds, respectively. Patients on active follow-up had 1.7 higher odds (95% CI 1.20-2.61; p = 0.004) and patients with higher levels of FCR scored lower on the global health status scale (72 vs. 83 p ≤ 0.001). Severe FCR is common in sarcoma survivors and high levels are related to a decreased global health status. FCR deserves more attention in sarcoma survivorship, and structured support programs should be developed to deliver interventions in a correct and time adequate environment.

Risk of subsequent primary lymphoma in a cohort of 69,460 five-year survivors of childhood and adolescent cancer in Europe: The PanCareSurFup study.

Survivors of Hodgkin lymphoma (HL) are at risk of developing non-Hodgkin lymphoma (NHL) after treatment; however, the risks of developing subsequent primary lymphomas (SPLs), including HL and NHL, after different types of childhood cancer are unknown. The authors quantified the risk of SPLs using the largest cohort of childhood cancer survivors worldwide. The Pan-European Network for Care of Survivors After Childhood and Adolescent Cancer (PanCare) Survivor Care and Follow-Up Studies (PanCareSurFup) cohort includes 69,460 five-year survivors of childhood cancer, diagnosed during 1940 through 2008, from 12 European countries. Risks of SPLs were quantified by standardized incidence ratios (SIRs) and relative risks (RRs) using multivariable Poisson regression. Overall, 140 SPLs, including 104 NHLs and 36 HLs, were identified. Survivors were at 60% increased risk of an SPL compared with the general population (SIR, 1.6; 95% confidence interval [CI], 1.4-1.9). Survivors were twice as likely to develop NHL (SIR, 2.3; 95% CI, 1.9-2.8), with the greatest risks among survivors of HL (SIR, 7.1; 95% CI, 5.1-10.0), Wilms tumor (SIR, 3.1; 95% CI, 1.7-5.7), leukemia (SIR, 2.8; 95% CI, 1.8-4.4), and bone sarcoma (SIR, 2.7; 95% CI, 1.4-5.4). Treatment with chemotherapy for any cancer doubled the RR of NHL (RR, 2.1; 95% CI, 1.2-3.9), but treatment with radiotherapy did not (RR, 1.2; 95% CI, 0.7-2.0). Survivors were at similar risk of developing a subsequent HL as the general population (SIR, 1.1; 95% CI, 0.8-1.5). In addition to HL, the authors show here for the first time that survivors of Wilms tumor, leukemia, and bone sarcoma are at risk of NHL. Survivors and health care professionals should be aware of the risk of NHL in these survivors and in any survivors treated with chemotherapy.

Fatal heart disease in patients with bone and soft tissue sarcoma.

Background/purposeWith improved cancer survivorship, non-cancer events, especially heart disease (HD), have become the underlying cause of death in cancer patients, but the risk of HD mortality in sarcoma patients remains poorly characterized. Therefore, our purpose was to: (1) identify sarcoma patients at the highest risk of fatal HD compared with the general population, (2) identify patients and sarcoma characteristics associated with a higher risk of HD death, and (3) determine if chemotherapy increased the risk of HD death in sarcoma patients.MethodsFrom 1975 to 2016, we identified patients diagnosed with bone and soft tissue sarcoma from the Surveillance, Epidemiology, and End Results (SEER) database in the US. Standardized mortality ratios (SMRs) were evaluated using mortality data from the general population collected by the National Center for Health Statistics. This was the largest retrospective cohort study of fatal HD in individuals with sarcoma.ResultsIn 80,905 sarcoma patients observed for 530,290 person-years, 3,350 deaths from HD were identified with a mortality of 631.7/100,000 person-years. The SMR of death from HD was 1.38 (95% CI: 1.33–1.42). The highest risks of death from HD were observed in patients with Ewing sarcoma (SMR = 5.44; 95% CI: 3.38–8.75) and osteosarcoma (SMR = 1.92; 95% CI: 1.55–2.38). Patients diagnosed at < 19 years old had the highest SMR in all age subgroups, and a higher risk of fatal HD relative to the general population was observed in sarcoma survivors diagnosed at < 85 years old. In patients diagnosed at < 19 years old, HD plurality occurred in those with Ewing sarcoma (29.4%) and osteosarcoma (32.4%) and at > 35 years old, HD plurality occurred in those diagnosed with liposarcoma (19.0%) and malignant fibro histiocytoma (MFH) (23.6%). For sarcoma survivors, HD mortality risks were highest within the first year after diagnosis (SMR = 1.31; 95% CI: 1.21–1.41), and this risk remained elevated throughout follow-up compared with the general population. Subgroup analyses indicated that chemotherapy significantly increased the risk of fatal HD in patients with localized osteosarcoma (Hazard ratio (HR) = 3.18; 95% CI: 1.24–8.13; P = 0.016), but not in patients with other histological sarcoma subtypes and clinical stages.ConclusionThe risk of death from HD mainly varied in patients with different histological sarcoma subtypes and clinical stages. Chemotherapy increased the risk of fatal HD in patients with localized osteosarcoma. To lower the risk of fatal HD in patients with sarcoma, we call for enhanced multidisciplinary cooperation, including cardiologists and orthopedic surgeons.

Impact of Limb Salvage on Prognosis of Patients Diagnosed With Extremity Bone and Soft Tissue Sarcomas.

BackgroundAlthough clinicians and patients with extremity bone and soft tissue (EBST) are increasingly interested in limb salvage surgery (LSS), because of the minimal damage to physical appearance and function, however, there is still a lack of large-scale population studies on whether LSS improves the prognosis of patients.PurposeThe aim of this study was to compare the survival of patients with EBST sarcomas after receiving LSS and amputation.MethodsTo conduct the population-based study, we identified 6,717 patients with a histologically diagnosed bone sarcoma and 24,378 patients with a histologically diagnosed soft tissue sarcoma from the Surveillance, Epidemiology, and End Results database. We analyzed overall survival (OS), cancer-specific survival (CSS), and non-sarcoma survival (NSS) using the Kaplan–Meier method, log-rank test or Gray test, Cox regression model, propensity score-matched analysis, and landmark analysis.ResultsLSS could improve the prognosis in patients with most EBST subtypes, except for Ewing sarcomas and MPNST. However, in the subgroup without distant metastases, limb salvage increased CSS only for patients with osteosarcoma, Ewing sarcoma, and leiomyosarcoma, as well as NSS for patients with chondrosarcoma and synovial sarcoma. Landmark analysis further demonstrated that sarcoma survivors surviving <10 years could benefit from LSS but not for long-term survivors ≥10 years. Moreover, for patients with distant metastases, LSS could improve survival of osteosarcoma patients but worsen CSS among patients with MPNST. Landmark analysis further demonstrated that LSS improved survival among osteosarcomas patients with distant metastases only within 1 year after surgery. Moreover, patients receiving LSS and those receiving amputation had a high risk of dying from different non-sarcoma diseases during the postoperative follow-up.ConclusionsThe impact of limb salvage on the prognosis of patients depends on the pathological subtype and stage of EBST sarcomas.

Improvements in life expectancy among childhood cancer survivors: Uneven gains and remaining challenges.

10050 Background: Childhood cancer survivors are at risk for shortened lifespan. Projections of life expectancy (LE) by diagnosis can provide benchmarks for assessing improvements over time. Methods: We developed a simulation model to project risk for common, life-threatening chronic health conditions (CHCs; heart failure, myocardial infarction, valvular disease, stroke, secondary breast cancer, colorectal cancer, glial tumors, sarcomas) and excess mortality among 5-year survivors, based on patient characteristics (sex, age at diagnosis, diagnosis) and treatment exposures (chemotherapy, radiation dose). Risk was estimated using statistical models and Childhood Cancer Survivor Study data. Age-related CHC risks (SEER, NHLBI) and competing mortality (CDC Wonder) were based on national databases. We used model calibration to identify parameter sets that generated outcomes consistent with observed data. Model outcomes included conditional LE and 10-year survival probability at age 40. For comparisons to the general population, we simulated age-, sex-, and diagnosis year-matched individuals who faced only competing mortality rates. Results: Among a cohort representative of the CCSS (mean diagnosis age, 7.4 yrs), compared to the general population, the gap in LE among survivors diagnosed in the 1970s vs. 1990s decreased from 17 yrs (25%) to 11 yrs (17%). Changes in LE among survivors diagnosed in the 1990s vs. 1970s varied by diagnosis, with leukemia, lymphoma, and CNS tumor survivors estimated to live an additional 8 to 11 yrs (Table). In contrast, considerably smaller gains were estimated for sarcoma and renal tumor survivors (1–3 yrs) and a loss for neuroblastoma (-3 yrs). Among survivors who reached age 40, the probability of surviving an additional 10 years increased from 89% to 92% between 1970s vs. 1990s, with the greatest gains for lymphoma and CNS tumors. Conclusions: Although temporal changes in pediatric oncology are projected to result in LE gains among survivors, considerable variation is projected across diagnoses. These findings highlight the uneven success of improving treatments for all cancers. [Table: see text]

Osteosarcoma/Ewing Sarcoma

Osteosarcoma/Ewing Sarcoma

Secondary malignant neoplasms after bone and soft tissue sarcomas in children, adolescents, and young adults.

Increased survival in young sarcoma patients comes along with a higher incidence of second malignant neoplasms (SMNs). The incidence, latency, histiotype, and outcome of these patients were analyzed because this information is essential to design evidence-based long-term follow-up care programs for young sarcoma survivors. Patients entered on clinical trials or registered in registries with a primary sarcoma in 1 of the cooperative sarcoma study groups in the framework of the Society for Pediatric Oncology and Hematology (GPOH) were screened for SMNs. Descriptive analysis, the Kaplan-Meier method, the Gray model, the Fine-Gray model, and the Cox regression model were used for the statistical analyses. A total of 159 out of 7079 (2.2%) patients were registered with a SMN. Among them, 104 solid SMNs (65%) and 56 hematologic SMNs (35%) occurred. Median latency from first diagnosis of sarcoma to the diagnosis of SMN was 6.8 years (range, 0-26.7 years). Cumulative incidence of SMN was 8.8% after 30 years. Five-year-survival was 67.1% (95% confidence interval [CI], 66.0-68.2) for the 7079 patients and it was 45.1% (95% CI, 36.2-53.6) after the diagnosis of a SMN (subcohort of n = 159 patients). There is a remarkable high cumulative incidence of SMNs after bone and soft tissue sarcomas in children, adolescents, and young adults. Therefore, effective transition as well as risk adapted long-term follow-up care programs should be developed and offered to young sarcoma survivors. Bone sarcomas and soft tissue tumors are rare tumors in children, adolescents, and young adults. The treatment varies, but may comprise chemotherapy, surgery, and/or radiotherapy. Developing a subsequent malignant tumor is a long-term risk for the patients. To better characterize this risk, we analyzed the data of 7079 patients (up to 21 years old) with bone sarcomas or soft tissue tumors. Our findings provide a basis to counsel young sarcoma survivors on their individual risk of subsequent malignant tumors. Moreover, these data can help to establish recommendations for aftercare in young sarcoma survivors.

Patient-reported neurocognitive function in adult survivors of childhood and adolescent osteosarcoma and Ewing sarcoma.

Little is known regarding long-term neurocognitive outcomes in osteosarcoma and Ewing sarcoma (EWS) survivors despite potential risk factors. We evaluated associations among treatment exposures, chronic health conditions, and patient-reported neurocognitive outcomes in adult survivors of childhood osteosarcoma and EWS. Five-year survivors of osteosarcoma (N = 604; median age 37.0years) and EWS (N = 356; median age 35.0years) diagnosed at < 21years from 1970 to 1999, and 697 siblings completed the Childhood Cancer Survivor Study Neurocognitive Questionnaire and reported chronic health conditions, education, and employment. Prevalence of reported neurocognitive difficulties were compared between diagnostic groups and siblings. Modified Poisson regression identified factors associated with neurocognitive difficulties. Osteosarcoma and EWS survivors, vs. siblings, reported higher prevalences of difficulties with task efficiency (15.4% [P = 0.03] and 14.0% [P = 0.04] vs. 9.6%, respectively) and emotional regulation (18.0% [P < 0.0001] and 15.2% [P = 0.03] vs. 11.3%, respectively), adjusted for age, sex, and ethnicity/race. Osteosarcoma survivors reported greater memory difficulties vs. siblings (23.5% vs. 16.4% [P = 0.01]). Comorbid impairment (i.e., ≥ 2 neurocognitive domains) was more prevalent in osteosarcoma (20.0% [P < 0.001]) and EWS survivors (16.3% [P = 0.02]) vs. siblings (10.9%). Neurological conditions were associated with worse task efficiency (RR = 2.17; 95% CI = 1.21-3.88) and emotional regulation (RR = 1.88; 95% CI = 1.01-3.52), and respiratory conditions were associated with worse organization (RR = 2.60; 95% CI = 1.05-6.39) for EWS. Hearing impairment was associated with emotional regulation difficulties for osteosarcoma (RR = 1.98; 95% CI = 1.22-3.20). Patient report of cognitive difficulties was associated with employment but not educational attainment. Survivors of childhood osteosarcoma and EWS are at increased risk for reporting neurocognitive difficulties, which are associated with employment status and appear related to chronic health conditions that develop over time. Early screening, prevention, and treatment of chronic health conditions may improve/prevent long-term neurocognitive outcomes.

"You either sink or you swim, and you're better off swimming": A qualitative study exploring the self-management experiences of soft tissue sarcoma survivors.

To qualitatively explore the consequences of soft tissue sarcoma and its treatment experienced by survivors, the self-management strategies they use to deal with these consequences, and any factors that may act as barriers or facilitators to their self-management. Semi-structured interviews were conducted with seven soft tissue sarcoma survivors who had completed their primary treatment. Interviews were audio-recorded, transcribed and thematically analysed. Physical, psychological and social consequences of soft tissue sarcoma and its treatment were identified, with side-effects, physical restrictions, body image issues, fear of recurrence, feeling depressed, familial relationships and sexual activity being the most frequently reported. Nine different types of self-management strategy encompassing eighteen specific strategies were identified, including cognitive strategies, lifestyle changes and utilisation of resources. Personal, social and environmental facilitators of self-management were identified; being in a relationship, being at an appropriate life stage, and having support from family, friends and medical staff were most commonly reported. Finally, personal and environmental barriers to self-management included the adaptation period, ongoing complications, rareness of sarcoma and poor patient-healthcare provider communication. The findings of this study suggest that soft tissue sarcoma survivors, especially those who are younger and experience physical limitations, may find the immediate post-treatment period particularly challenging and may benefit from nurse-led self-management support.