Quality of life and Q-TWiST were not adversely affected in Ewing sarcoma patients treated with combined anlotinib, irinotecan, and vincristine: (Peking University People's Hospital Ewing sarcoma trial-02, PKUPH-EWS-02).

BACKGROUND: Combined treatment with anlotinib, irinotecan, as well as vincristine for advanced Ewing sarcoma (EWS) has been verified been effective in the prospective trial of Peking University People's Hospital EWS trial-02. We aimed to assess the dynamic changes in health-related quality of life (QoL) and the benefit-risk in quality-adjusted survival in current study. METHODS: Twelve "pediatric" patients and 23 "adult" patients were enrolled. QoL was assessed with the EORTC QLQ-C30 for adults and PedsQL 3.0 Cancer Module for children and adolescents. The quality-adjusted time without symptoms of disease progression or toxicity of treatment (Q-TWiST) analysis was used to describe treatment results. RESULTS: Progression-free survival was not accompanied by diminished QoL. Differences in scores on the QoL global health status and specific functioning before, during, and after treatment were not significantly different with time (P = .14 for adults and .91 for children). During treatment, there was a statistically insignificant trend towards improved QoL with reduced tumor burden (P = .14 for adults and .10 for children), but QoL significantly declined with progression of disease (P = .05 for adults and .04 for children). The most common adverse events were neutropenia (12.1%), leukopenia (16.6%), anemia (12.7%), and diarrhea (4.93%). Results across the trial analyses showed that the median time of Q-TWiST was 0.73 (interquartile range, 0-1.57) months, whereas the median time with toxicity before disease progression was 3.9 (interquartile range, 2.3, 6.1). CONCLUSION: QoL exhibited a trend towards improvement in accordance with high objective response in this trial with the receipt of combination therapy of anlotinib, vinsristine, and irinotecan for advanced EWS. The toxicity profile did not translate into significantly worse overall scores during treatment.

Parents Demand and Teenager Refuses Epidural Anesthesia.

A 15-year-old girl is scheduled to undergo an upper lobectomy to debulk metastatic Ewing sarcoma. The anesthesiologist recommended placement of a thoracic epidural catheter to provide postoperative analgesia. The patient did not want a needle to be placed near her spine. She was terrified that the procedure would be painful and that it might paralyze her. Although the anesthesiologist reassured her that sedation and local anesthesia would make the procedure comfortable, she remained vehemently opposed to the epidural procedure. The parents spoke privately to the anesthesiologist and asked for placement of the epidural after she was asleep. They firmly believed that this would provide optimal postoperative analgesia and thus would be in her best interest. Experts discuss the pros and cons of siding with the patient or parents.

Identifying bone sarcoma survivors facing psychosocial challenges. A study of trajectories following treatment.

OBJECTIVE: Bone sarcoma survivors face a number of physical and psychosocial challenges in relation to the late effects they experience following treatment. The present study aimed to identify and explore the different trajectories that bone sarcoma survivors might navigate during follow-up. METHODS: In-depth and semi-structured interviews were conducted, and an inductive thematic analysis was performed. RESULTS: When they were interviewed three to ten years after the primary diagnosis, the eighteen bone cancer survivors were found to be in three different rehabilitation phases that followed fairly distinct trajectories, namely, back to normal, a new normal and still struggling. Only three participants felt that they had returned to a life that was quite similar to the one they had lived prior to having cancer. Fifteen participants considered their lives and their bodies to be significantly altered. CONCLUSION: Sarcoma survivors who undergo life-changing treatment and return to very different lives than they had before should be identified by healthcare professionals and guided through this demanding phase to better cope with their new living conditions. Information on and tailored guidance related to psychosocial challenges may be of particular importance. Active focus on reorientation, as well as possibilities for growth, seems to be important.

Patient perspectives on a national multidisciplinary team meeting for a rare cancer.

Multidisciplinary team meetings (MDTM) provide a regular forum for cancer teams to convene and discuss the diagnostic and treatment aspects of patient care. For some rare cancers, MDTMs may also occur at national level to pool expertise and to ensure more consistent decision-making. One such national MDTM exists in the UK for patients with a diagnosis of Ewing's sarcoma of the bone-the National Ewing's MDT (NEMDT). This study explored the patient perspective of this rare cancer national MDTM using focus group and survey methodology. Study participants used their experience to provide several recommendations: that their views should always inform the decision-making process, these views should be presented by someone who has met them such as a specialist nurse, MDT recommendations should be provided to them in plain English, and tools to improve patient choice and enhance communication should be implemented. These patient-centred recommendations will be used to improve the NEMDT but may be valid to inform quality improvement processes for other similar national panels.

Uso de biofeedback de variabilidad de la frecuencia cardiaca durante la radioterapia como método de distracción cognitiva y autorregulación en un paciente pediátrico: Informe de caso / Biofeedback use of heart rate variability during radiotherapy as a method of cognitive distraction and self-regulation in a pediatric patient: A case report

Objetivo: Evaluar la efectividad del uso de biofeedback de variabilidad de frecuencia cardiaca (VFC) durante la radioterapia como método de distracción cognitiva y autoregulación emocional en un paciente pediátrico. Método: La paciente es una niña de 11 años con diagnóstico de sarcoma de Ewing en la pierna izquierda. Recibió 28 sesiones de radioterapia. Antes de iniciar tratamiento, se evaluó psicológicamente a la paciente con la entrevista Mini Kid, identificando que no tenía psicopatología. Dos semanas previas a iniciar, se evaluó la frecuencia cardiaca (FC) y variabilidad (VFC) de la misma (indicadores que han sido utilizados previamente como correlatos fisiológicos de estrés y relajación) con un software especializado llamado EmWave, identificando que sí presentaba activación fisiológica asociada a estrés. Se hizo entrenamiento en respiración diafragmática apoyado con el equipo de retroalimentación dos semanas previas a la radioterapia. El día que inició tratamiento, se volvió a evaluar la frecuencia y variabilidad antes de comenzar. Durante la radioterapia se instrumentó a la paciente al software y se permitió que ella escogiera el juego (que registra las variables de FC y VFC e interactúa conforme ella logra autoregulación fisiológica) y éste era proyectado en el techo de la sala mientras recibía la radiación. Resultados: Se identificaron cambios en la variabilidad de la frecuencia cardiaca, logrando una autoregulación, mostrando así que la paciente aprendió a relajarse y que lo puso en práctica durante la radioterapia. Conclusión: El uso del biofeedback es innovador en el tratamiento con radioterapia; permite al paciente integrar los dos métodos más utilizados como preparación para la misma: distraer el foco atencional y lograr una autoregulación. Se sugiere ampliar la muestra para conocer los efectos y generalizar los resultados (AU)

Aim: To assess the effectiveness of heart rate variability (HRV) biofeedback during radiotherapy as an intervention to promote cognitive distraction and emotional regulation un a paediatric patient. Method: Patient is an 11 years old female with Ewing Sarcoma on left leg. She recieved 28 radiotherapy sessions. Before initiating treatment, the patient was assessed with Mini International Neuropsychiatric Interview KID (MINI KID). She did not have a psychiatirc diagnostic, nor significative anxiety as rated by these instruments. Two weeks prior initiating radiotherapy, Heart rate (HR) and heart rate variability (HRV) (measures that have been used previously as physiologic corralates of stress and relaxation) were measured with a specialized software called EmWave. Results identified that there was a psysiologic activation related to stress. At the same session, the patient was trained in diaphragmatic breathing assisted with HRV biofeedback. The day the radiotherapy treatment started, HR and HRV were assessed an hour before. During radiotherapy, patient was instrumented to the software and the display was projected on the ceiling so that she could interact with the program at the same time she recieved radiotherapy. Patient was asked to choose the display she prefered (presented as interactive games by EmWave) and HR as well as HRV were measured during treatment. Results: Changes in HRV during radiotherapy were presented, these changes have been reported as indicative of self regulation, reflecting that the patient learned this behavior and was able to implement it during her treatment. Conclussion: The use of biofeedback is innovative in treatment with radiotherapy; it allows the patient to integrate the two most used methods for psychological preparation: attentional distraction and self regulation methods. It’s suggested to amplify the sample in order to learn more about the use of biofeedback in radiotherapy and generalize results (AU)

IT-based Psychosocial Distress Screening in Patients with Sarcoma and Parental Caregivers via Disease-specific Online Social Media Communities.

BACKGROUND: Psychosocial distress can be frequently observed in patients with sarcoma, depicting a relevant clinical problem. However, prospective data collection on psychosocial distress in patients with rare tumors is often time-consuming. In this context, social media such as Facebook can serve as a potential platform to expand research. The aim of this study was to assess the feasibility of psychosocial distress screening in patients with osteosarcoma and Ewing's sarcoma via social media. MATERIALS AND METHODS: For this study an online questionnaire including general information and self-assessment distress measurement tools for patients and parents was created. The link to the questionnaire was then posted on the main page of the two largest disease-specific Facebook communities on osteosarcoma and Ewing's sarcoma. RESULTS: Within 2 months, 28 patients and 58 parents of patients were enrolled. All patients with osteosarcoma and Ewing's sarcoma, as well as the majority of parental caregivers of such patients, showed relevant psychosocial distress levels. CONCLUSION: Crowdsourcing via disease-specific patient communities on Facebook is feasible and provides great potential for acquisition of medical data of rare diseases.

Quality of Survivorship in a Rare Disease: Clinicofunctional Outcome and Physical Activity in an Observational Cohort Study of 618 Long-Term Survivors of Ewing Sarcoma.

Purpose Significantly improved survival rates in patients with Ewing sarcoma have raised interest in accessing the quality of long-term survivorship. In this study, subjective and objective measurement tools, preclassified as physical or mental scores, were used to assess clinicofunctional outcome and physical activity after intensive bone tumor treatment. Methods Long-term outcome of 618 survivors from consecutive Ewing sarcoma trials was assessed by the Toronto Extremity Salvage Score, Short-Form Health Survey (SF-36), Brief Symptom Inventory (BSI), and Rosenberg Self-Esteem Scale questionnaires and by the accelerometric StepWatch 3 Activity Monitor. Prospective measurements were correlated retrospectively with standardized primary trial data. Results were compared with 316 nonrandom healthy peers by using effect sizes ( d). Median observation time was 12.9 years from primary diagnosis (range, 3.7 to 31.2 years). Results Absolute subjective scores were moderate to good for survivors. Compared with control subjects, unfavorable outcome was shown on physical Toronto Extremity Salvage Score, SF-36 Physical Component Summary, and BSI-Somatization scales (| d| ≥ 0.50; P < .01), in contrast to SF-36 Mental Component Summary, BSI-Anxiety, BSI-Depression, and Rosenberg Self-Esteem Scale mental scales (| d| ≤ 0.31). Survivors were less active than control subjects, as demonstrated by a step count difference of 1,742 steps per day ( d = -0.43; P < .01); however, on average, the recommended level for an active lifestyle was achieved (≥ 10,000 steps). Location of pelvic tumor was the major inferior disease-specific prognostic factor in physical scores ( P < .01), whereas nondisease-specific inferior factors in questionnaires were older age and female sex ( P < .01). Conclusion Survivors of Ewing sarcoma apparently returned to a normal life with minor limitations. Observed reductions in physical scores should be a focus in future research to optimize treatment strategies to reduce a negative impact on the quality of survivorship.

Sarcoma survivors' perspectives on their body image and functional quality of life post-resection/limb salvage surgery.

Sarcomas often occur in patients' extremities and treatment typically involves bone resection/limb salvage surgery. Such treatments leave survivors with physical disfigurements, functional disabilities, and/or emotional traumas. Our post-surgery psychological intervention investigated how these experiences impinge on sarcoma survivors' lives. Twenty-three survivors aged 19-60 years (M = 36 years) participated in a tri-disciplinary (rehabilitative exercise, plastic surgery and psychological) intervention. Of these, 17 participated in psychodynamic counselling, 10 completed a mental-health questionnaire and seven kept a reflective journal. An exemplar case study research design was employed and data were subjected to interpretative phenomenological analysis. The findings reveal that survivors typically experience a number of body image issues and mobility difficulties, which they are reluctant to share with their oncologist in case they are viewed as being ungrateful or vain. In instances where such issues remain unaddressed, then sarcoma survivors have a tendency to adopt avoidant coping strategies and social isolation practices. These practices negatively impact on their mental health and functional quality of life. Hence, it is suggested that a short three part (body image, mobility, and coping strategy) screen be devised and used at all sarcoma 2-year follow-up assessment consults to identify which survivors are in need of psychological assistance.

Health related quality of life and late side effects of long-term survivors of Ewing's sarcoma of bone.

PURPOSE: The data examining the Health Related Quality of Life (HRQOL) after definitive treatment for Ewing's sarcoma (EWS) is sparse. The objective of this study was to assess the HRQOL and late side effects in EWS of bone survivors treated in the past 2 decades. METHODS: Seventeen long-term (≥ 5 years) EWS survivors (age range, 11-27 years) treated from 1990 to 2004 completed the EORTC-QLQ-C30. We compared the HRQOL of our data set with a reference group of cancer survivors and also with the general population. Musculoskeletal late treatment toxicity was also evaluated. The Mann-Whitney and Wilcoxon tests were used for analyses. RESULTS: EWS survivors of our series reported significantly better global, physical, role and cognitive HRQOL and less symptoms of fatigue, pain and insomnia than the normative sample of cancer survivors. Moreover, the HRQOL was equivalent to the general population, with the exception of social functioning. Additionally, patients with tumor location in the extremities did not report significant HRQOL differences compared with those with tumor at other locations. Common chronic grade 2 side effects were generalized muscle weakness (23%) and decreased joint range of motion (23%). Only one patient experienced musculoskeletal chronic grade 3 toxicity. CONCLUSION: Our findings suggest that EWS survivors treated in the modern era do not experience lower HRQOL than other cancer survivors. Rather, the HRQOL appears to be equivalent to the general population. Further and larger studies are needed to confirm these results.

Metabolic syndrome traits in long-term survivors of pediatric sarcoma.

PURPOSE: The metabolic syndrome (MS), a cluster of central obesity, dyslipidemia, hyperglycemia, and hypertension, conveys an increased risk of type 2 diabetes and cardiovascular disease. This cross-sectional study investigated the prevalence of metabolic syndrome traits (MST) in long-term survivors of pediatric sarcoma (SARC) who received multi-modality therapy (MMT). METHODS: Thirty-two SARC survivors (predominantly Ewings; median age 36.5; median age at MMT 15) underwent body composition, activity, and psychosocial analysis. Serum endocrine and inflammatory parameters and urine beta(2)-microglobulin (B2M) were evaluated. The prevalence of MST was compared to age- and gender-matched U.S. population data. RESULTS: SARC survivors were more likely to have two or more MST (OR 2.38 95% CI: [1.14, 5.04]). Analysis of individual MST demonstrated higher prevalence of hypertension (OR 2.61 95% CI: [1.20, 5.59]), hypertriglyceridemia (OR 3.63 95% CI: [1.75, 7.60]), and male visceral abdominal obesity (20-39 years old OR 4.63 95% CI: [0.91, 21.63], 40-59 years old OR infinity). Survivors 18-39 years old had a higher prevalence of the MS (OR 4.29 95% CI: [1.50, 11.21]), defined as three or more MST. Plasminogen activator inhibitory activity (P = 0.016) and B2M (P = 0.027) increased with increasing numbers of MST. In males, total testosterone declined (P = 0.0027) as the number of MST increased. Average (P = 0.014) and maximum (P = 0.021) activity levels decreased as the number of MST increased. CONCLUSION: After a median follow up of 17 years, adult SARC survivors of MMT had an increased prevalence of MST, especially those less than 40 years old. The development of MST in this population was associated with decreased testosterone and activity levels.

Achieving balance: a case study examination of an adolescent coping with life-limiting cancer.

A single-case study approach was used to provide an in-depth examination of the special events that take place in an early adolescent's family as the adolescent attempts to adapt to living with life-limiting cancer. Through symptom control, hope, denial, and the achievement of developmental tasks such as peer group identification, independence, and autonomy, the early adolescent was able to demonstrate positive coping and adaptation. In the context of the Calgary Family Systems Model, the adolescent achieved individual system balance, which in turn enabled his family's systems to balance. The health care professional played an integral role in enabling the adolescent to accomplish his developmental tasks, which translated into facilitating adaptation.

Surveillance of second cancer after previous childhood cancer treatment.

BACKGROUND: With the introduction of new therapeutic strategies, the survival of children and adolescents with cancer has increased dramatically. However, cancer survivors often experience late effects from their cancer treatment. OBJECTIVE: A case study is presented that highlights the wide range of issues that may be encountered in young cancer survivors and underscores the necessity of continued follow up in this group of patients. DISCUSSION: Risk factors and the recommended surveillance of second cancer in this group of patients are discussed.

Quality of life of patients treated during childhood for a bone tumor: assessment by the Child Health Questionnaire.

To determine which factors impact on quality of life of patients with bone tumor, we used the Children Health Questionnaire French version. Thirty-seven patients (25 males, 19 osteosarcoma, 18 Ewing sarcoma) were studied. At assessment, median age was 15 years, median follow-up was 4 years. Mean scores were 60, 81, 76, 74, 70, 87 for general health, physical functioning, pain, mental health, self-esteem, and family activity, respectively. Lower results were observed for mental health in girls, for physical functioning, and self-esteem in patients with endoprosthesis, and for family activity and pain in patients who had relapsed.