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.

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.

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.

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.

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.

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 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.

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.

Neuropsychological outcome in long-term survivors of a childhood extracranial solid tumor who have undergone autologous bone marrow transplantation.

The purpose of this study was to evaluate neuropsychological and adaptive functioning of children who have undergone bone marrow transplantation (BMT) without previous cranial irradiation. In total, 76 children treated for an extracranial tumor with BMT without total body irradiation (TBI) were evaluated at least 5 years after the end of the treatment.Overall, their performance and skills were in the normal range and their professional and academic outcomes were satisfactory. Nevertheless, we observed a deleterious effect of deafness on verbal IQ associated with the previous administration of cisplatin during conventional chemotherapy. In addition, reading difficulties had arisen. This could be related to absence from kindergarten or primary school during hospitalization. Finally, in the younger subgroup, visual-perceptual skills were found to be more fragile.

An autistic boy copes with a terminal illness.

David, a 12-year-old boy with autism, developed Ewing's Sarcoma and died 3 months later. This report describes the effects of both illnesses on David, his parents, and his care givers. The difficulties encountered in finding appropriate help in the care of both illnesses and in making difficult decisions are described. The presence of autism can affect medical management and the presence of terminal illness can affect the management of autism. Suggestions are offered for effective medical and psychiatric interventions that include a sensitivity to both illnesses in physicians and other care givers; a case manager to help find appropriate medical and psychiatric services; and the availability of combined medical and psychiatric units.

Experiences of cancer in children and adolescents.

In an effort to understand the effect of cancer diagnosis and treatment in children and adolescents, and to identify issues that should be addressed with newly diagnosed patients, 85 patients with Ewing's sarcoma family tumors (ESFT) were interviewed about their experience of having cancer. This represents 90% of all eligible patients who survived at least 3 years since their diagnosis and who were treated for ESFT at the National Cancer Institute (NCI) from 1965-1993. The mean age of patients at the time of diagnosis was 15.8 +/- 5.3 years, and mean time since diagnosis was 13.6 +/- 6.4 years. Patients from this cohort had a disease usually related to poor outcome. Patients answered five open-ended written questions. Negative experiences that they described included transient and permanent discomfort and disabilities related to cancer; disruption of life or relationships; and emotional aspects of cancer diagnosis or treatment. Positive aspects of having cancer included changed attitudes about self and life, improved relationships with others, or better job performance. Advice for newly diagnosed patients most often dealt with the emotional aspects of cancer. The importance of patient-to-patient support was frequently described. Overall, having cancer was not an entirely negative experience, and it may result in introspection and improved relationships with others.

Clinical experience with psychological aspects in pediatric patients amputated for malignancies.

AIMS AND BACKGROUND: Amputation surgery in pediatric patients suffering from malignant tumors is less common than in the past, but has a great emotional impact on patients and their families as well as on the medical team. Studies addressing the psychological aspects of limb amputation in childhood cancer are still relatively limited, and the results have sometimes been contradictory. METHODS: At the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan psychological support was provided to candidates for amputation and their families, involving medical oncologists, a clinical psychologist, and social assistants. Twenty-two patients were analyzed and 16 underwent mutilating surgery. RESULTS: Different emotional reactions were observed. Surgery proved to be easier to accept when the tumor caused pain and functional loss. Specialist medical psychological support was needed in case of defense mechanisms (eg, splitting and projection) and depressive reactions evolving into isolation or intolerance. CONCLUSIONS: The reported experience could be helpful in providing adequate support to children with tumors requiring mutilating surgery.