Pediatric sarcoma survivorship: A call for a developmental cascades approach.

Survivors of pediatric sarcomas often experience greater psychological and psychosocial difficulties than their non-afflicted peers. We consider findings related to poorer outcomes from a developmental cascade perspective. Specifically, we discuss how physical, neurocognitive, psychological, and psychosocial costs associated with pediatric sarcomas and their treatment function transactionally to degrade well-being in long-term pediatric sarcoma survivors. We situate the sarcoma experience as a broad developmental threat - one stemming from both the presence and treatment of a life-imperiling disease, and the absence of typical childhood experiences. Ways in which degradation in one developmental domain spills over and effects other domains are highlighted. We argue that the aggregate effect of these cascades is two-fold: first, it adds to the typical stress involved in meeting developmental milestones and navigating developmental transitions; and second, it deprives survivors of crucial coping strategies that mitigate these stressors. This position suggests specific moments of intervention and raises specific hypotheses for investigators to explore.

Viviendo con sarcoma maligno en miembros inferiores: experiencia adolescente / Living with malignant sarcoma in lower limbs: adolescent experience

Padecer cáncer se considera una situación devastadora que divide la vida de quien lo padece y genera cambios a nivel físico, mental, emocional, social y espiritual. Entre las neoplasias malignas, se encuentran el osteosarcoma y el sarcoma de Ewing como los dos tipos de sarcomas más representativos en la adolescencia y en ubicación anatómica en miembros inferiores. Los adolescentes que padecen de sarcoma se enfrentan a una situación que amenaza su vida o su integridad y que supera todas las herramientas con las que cuentan para enfrentarla, lo cual los lleva al límite y les genera una crisis vital. Esta situación ha sido ampliamente abordada por diferentes investigaciones desde el análisis de la fisiopatología, el diagnóstico, el tratamiento, la rehabilitación; mientras que otras estudian el manejo de los síntomas, la calidad de vida y las vivencias de los adolescentes desde la visión de los adultos. Sin embargo, son escasos los estudios que abordan la experiencia de vida de los adolescentes con sarcoma maligno en miembros inferiores desde su propia voz. Objetivo: Comprender las experiencias de los adolescentes que viven con sarcoma maligno en miembros inferiores. Método: Estudio cualitativo, desarrollado bajo la metodología de teoría fundamentada desde el paradigma constructivista con once adolescentes entre los 10 y los 19 años que padecen de sarcoma en miembros inferiores. La recolección de los datos se llevó a cabo a través de cuatro métodos: entrevistas intensivas, análisis de redes sociales por medio de observación virtual participante, escritos y la investigadora. El análisis de los datos se realizó por medio del programa de ATLAS.ti, versión 8.0, a partir de los procesos de codificación inicial, enfocada y teórica, propuestos por Kathy Charmaz. La coconstrucción de la compresión de las experiencias se realizó entre los participantes como expertos y la investigadora, para lo cual se siguió una rigurosa comparación constante de los datos y su triangulación. Resultados: La comprensión de las experiencias de los adolescentes con sarcoma en miembros inferiores se representan en el planteamiento teórico Anteponiéndome al cáncer en mi pierna: de la invulnerabilidad a la emancipación. La trayectoria de las experiencias se enmarcó en tres categorías que fueron analizadas conforme avanzaba la trayectoria de la enfermedad, a saber: Dejando mi ser invulnerable: enfrentando mi nueva realidad; Persistiendo en vivir: aceptando, luchando y resistiendo; y Tomando las riendas de mi vida: ajustando y viviendo mi nueva vida. Finalmente, del proceso de comparación constante de las categorías emergió el planteamiento teórico a partir del cual, al ser contrastado con la literatura precedente, se definieron los siguientes seis conceptos centrales de la teoría sustantiva resultante: Vulnerabilidad; Motivación; Apoyo social; Afrontamiento; Resiliencia; y Emancipación. Conclusiones: La construcción de la experiencia de los adolescentes con sarcoma en miembros inferiores se consolidó dada la transición de dejar la percepción de invulnerabilidad de los adolescentes hasta el desarrollo de su emancipación. Esta emancipación, más allá de significar la separación de su núcleo familiar, implicó la diferenciación de los adolescentes como seres autónomos, empoderados, adaptables, motivados, resilientes, conscientes del apoyo social con el que cuentan, capaces de elaborar decisiones y conscientes no sólo de su propia vulnerabilidad, sino también de sus capacidades de afrontar las crisis vitales venideras.

Having cancer is a devastating situation that splits the life of those who suffer it, causing changes on a mental, physical, emotional, social, and spiritual levels. This is the case of sarcoma, a low incidence but high mortality malignant neoplasm. This type of tumor is more frequent in the lower limbs than in the upper limbs and adolescents are the main population at risk. Teenagers then face a life-threatening situation that takes them to the limit of their underdeveloped troubleshooting skills, which then provokes a life crisis. Such a situation has been approached from distinct fields of knowledge, some of them focused on the diagnosis and treatment of the disease, whilst others focus on quality of life and experience of the adolescent. Nonetheless, research surrounding life experiences of adolescents with malignant sarcoma of the lower limbs addressed from the perspective of the adolescent are scarce. Objectives: To understand the life-experience of adolescents that live with malignant sarcoma of the lower limbs. Methods: A qualitative grounded theory-based study was made from a constructivist paradigm. Eleven Adolescents aged 10 to 19 years old diagnosed with lower limb sarcoma participated in the study through intensive interviews, participating virtual observation and optional written records. The latter went through content analysis while the participating virtual observation was made through social network analysis and the researchers own reflection; interviews on the other hand were analyzed through ATLAS.ti 8.0 software following initial, focused, and theoretical coding proposed by Kathy Charmaz. Results: The comprehension of the experiences of adolescents with malignant sarcoma of the lower limbs is comprised in the theoretical approach called Overcoming cancer in my leg: From invulnerability to emancipation which is composed of three main categories that develop as the treatment of the disease takes place and represent the course of experiences endured by the adolescents. These three categories are Renouncing my invulnerability, facing my new reality; Persisting in living: accepting, fighting, and resisting; and Taking the reins of my life: adjusting and living my new life. From the constant comparison and contrast of these categories with precedent literature, six concepts are embraced for the theoretical approach, as follows: Vulnerability; Motivation; Social support; Coping; Resilience; and Emancipation. Conclusions: The construction of the experience of adolescents with lower limb sarcoma has revealed the development of a process of anticipated emancipation based on motivation, social support, coping and resilience. Such emancipation goes beyond the separation from the family nucleus and implies the differentiation of adolescents as autonomous, empowered, adaptative, motivated, resilient, and decision-making beings, aware of their own vulnerability and their capacity to endure upcoming vital crises that may arise.

Symptoms and their implications on quality of life and psychological distress in sarcoma patients.

The aim of this study was to investigate symptoms, their variation over time and their relationship with quality of life (QoL)/psychological distress in sarcoma patients, as few data regarding QoL and psychological distress in this set of patients are currently available. A total of 188 sarcoma patients from an Italian referral center were involved. Symptoms and financial difficulties were evaluated with the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire from the first treatment and over the follow-up period, up to 6 years. The authors found that patients with sarcoma experience several symptoms, especially fatigue and pain, which may dramatically worsen QoL and psychological distress. In conclusion, patients with sarcoma often experience fatigue, pain and financial difficulties, which negatively impacts QoL and psychological distress. To ameliorate overall QoL, proper control of symptoms is necessary.

Patient-Reported Outcomes in Sarcoma: A scoping review.

PURPOSE: Sarcoma is a heterogeneous group of tumours, usually affecting young patients and related to both endogenous and exogenous risk factors. The importance of obtaining the patient's perspective of the illness experience is imperative. Patient-reported outcomes (PROs) are the outcomes that come directly from the patient. They include symptoms, functional health, well-being, quality of life, psychological issues, among other indicators reported by the patients. The objective of this scoping review was to map the PROs in sarcoma patients and how they are measured. METHODS: The review process was guided by the Joanna Briggs Institute (JBI) checklist for scoping reviews. RESULTS: The search identified 116 potentially relevant studies, with 27 articles meeting the inclusion criteria. The most common PRO evaluated in the selected studies were health-related quality of life (HRQoL), followed by functional outcome, aspects of mental health, and specific symptoms. Generic HRQoL questionnaires were widely used. Quantitative studies usually applied more than one type of Patient-Reported Outcome Measures (PROMs) to measure different PROs. CONCLUSIONS: PROs should be carefully analysed to better understand the sarcoma patient's needs. The PROMs used in the selected studies about sarcoma were not specific to sarcoma, therefore, to better reflect on the perceptions of sarcoma patients, a different new and specific measurement strategy should be considered.

Retrospective quality of life study in patients with retroperitoneal sarcoma in an Asian population.

BACKGROUND: Retroperitoneal sarcoma represents 15% of sarcomas. The mainstay of treatment is surgery where a majority of patients require multi-visceral resections that may significantly impact their quality of life (QOL) following surgery. Studies in other cancers have shown that QOL may not be significantly impacted after radical or extensive surgery. However, there are limited studies examining the QOL specifically in patients with retroperitoneal sarcoma. In this pilot study, we retrospectively evaluated the QOL of patients with retroperitoneal sarcoma. METHODS: 32 out of 90 patients who underwent surgical intervention for retroperitoneal sarcoma in National Cancer Centre Singapore from January 1999 to August 2018 who were alive and on follow-up were included in this study. EORTC-QLQ-C30 was administered to the patients. RESULTS: The median age of our patients was 59 years (range, 35-84), and median time from surgery to the implementation of questionnaire was 2.5 years (range, 0.05-9.6). Younger patients had significantly better differences in global health, physical and role functioning scores as compared to older individuals. Female patients reported higher global health, physical, emotional and social functioning scores than males. Patients who were more than 2 years post-surgery exhibited better QOL scores as compared to those who had more recent surgery. Our patients had comparable global health and functioning scores compared to a reference group of outpatient cancer patients at our institution. CONCLUSIONS: Our pilot study investigating the QOL of patients with retroperitoneal sarcoma has shown that patients need to be followed up for at least 2 years following surgery to evaluate their QOL. In general, they achieved better functioning scores when compared with other cancer patients. These findings support the need for larger-scale prospective studies to further evaluate the QOL of these patients.

The unmet needs of patients with sarcoma.

OBJECTIVE: Sarcoma is a rare cancer that imposes a significant burden on the lives of patients. Many survivors have long-term disability as a result of treatment and the disease. Patients often experience functional issues, poorer mental health, reduced quality of life, and interpersonal issues. There is a need to explore the unmet needs of sarcoma patients as it is unclear how these issues are being addressed. The aim of this study was to explore the unmet needs of patients who have been diagnosed with sarcoma. METHODS: The participants were individuals previously diagnosed with a sarcoma (n = 22). Participants completed a semi-structured interview about their unmet needs relating to sarcoma. The transcripts were analyzed using thematic analysis. RESULTS: Five overarching themes were identified: daily living, financial needs, lack of information, need for a community, and navigating the healthcare system. Participants reported a range of practical needs, including transport, limited understanding of their treatments, and financial issues. Participants also described a need to connect with people who had gone through similar experiences. CONCLUSIONS: Sarcoma patients have fundamental needs that affect their capacity to live their life in the best possible way. Health services need to better facilitate sarcoma patients at each stage of their experience by providing individualized referrals, support, and coordination. Support interventions are needed to help patients adjust to sarcoma and to improve their quality of life as an outpatient. Connecting patients with sarcoma support groups may provide them with more relevant and intimate support.

Development of a patient-reported experience questionnaire for patients with sarcoma: the Sarcoma Assessment Measure (SAM).

PURPOSE: The aim of the study was to develop a patient-reported outcome measure for patients with sarcoma-the Sarcoma Assessment Measure (SAM). METHODS AND RESULTS: The systematic development of SAM included a three-stage, mixed-methods study using semi-structured interviews, focus groups and questionnaires, with all stages involving patients from across the United Kingdom. In-depth interviews were conducted with 121 patients (50% male; aged 13-82; with soft tissue sarcoma (62%), bone tumours (28%) and gastrointestinal stromal tumours (10%)). Content analysis of the interview transcripts identified 1415 post-diagnosis experience statements. Experience statements were reviewed, repetition was removed and sentences were refined to form 395 'items' which were included in an Item Reduction Questionnaire (IRQ) grouped as physical, emotional, social and financial wellbeing and sexuality. The IRQ was completed by 250 patients who rated each item on importance and worry. Items with a mean score above 5 (6 in the emotional domain) were removed, which reduced the list to 166 items. After review by the research team, 23 clinicians and 34 patients, 66 items were retained to test content validity. Items with a content validity ratio of < .33 were removed. Cognitive interviews were conducted with 10 patients on the final 22 items to test comprehension. Minor changes were made to four. CONCLUSION: SAM comprises of 22 items reflecting physical, emotional, social, financial wellbeing and sexuality. This systematic process of using patient experience to develop the content of SAM will ensure that it measures what is important to patients.

Psychological functioning, coping styles and their relationship to appraisal of physical limitations following invasive surgical procedures for soft-tissue sarcoma: A qualitative study.

BACKGROUND AND OBJECTIVES: This study explored psychological functioning and coping styles in adult patients with soft-tissue sarcoma who underwent surgical procedures in a single expert sarcoma medical center in Canada. METHODS: This is a qualitative study with three formats of data collection. The interview guide was based on theoretical health-related quality of life model. We began the investigation with 2 online and 2 in-person focus groups. Four individual semistructured interviews were added to further explore emerging themes. Data were analyzed using inductive thematic networks approach. RESULTS: Twenty-eight adults (13 female, 24-75 years of age) participated. In the domain of psychological functioning we identified three main themes; changes in mood, worry, and body image concerns. In the domain of coping styles, we identified four adaptive coping styles; positive reframing and optimism, finding a purpose, being proactive, and using humor. Among the maladaptive coping styles, we found passive acceptance, and avoidance and denial. CONCLUSIONS: Psychological well-being can be contingent on physical functioning and coping styles in adults with soft-tissue sarcoma. Both psychological and physical function impact quality of life. Patients with more physical limitations, psychological distress and maladaptive coping styles should be monitored for their well-being.

Experiences of return to work after treatment for extremital soft tissue or bone sarcoma: Between distraction and leaving the disease behind.

OBJECTIVE: We aimed to explore the return to work (RTW) experience of individuals in remission from extremital sarcoma. METHODS: Using a qualitative survey design, we asked sarcoma survivors about their RTW experiences after treatment. Seven men and eight women (n = 15), 43 years old in average, participated. The majority had soft tissue sarcoma (n = 14) and no amputations (n = 14). We analysed data thematically through an inductive approach. RESULTS: Participants' motivation to RTW and their experiences of this transition could be understood under the main theme of "searching for distraction and wanting to leave the disease behind," followed by "problems of the new normal." RTW was the next step after treatment; however, being back at the same workplace/institution was challenging due to the interference of the effects of the disease and treatments and peer perceptions. In other subthemes, we identified that RTW was facilitated by different "signs of readiness," as well as by "motivating factors and the meaning of having an occupation." CONCLUSIONS: Readiness to RTW while primarily an autonomous decision can also be guided by third parties, including treating physicians. RTW can be challenging, since changes in performance as well as comments from peers become a constant reminder of the status prior to the illness. Given that RTW helps distract from the disease, these reminders can be confronting and highlight the role of health professionals and employers in preparing survivors and peers to facilitate the RTW.

Validation process of Toronto Exremity Salvage Score in Italian: A quality of life measure for patients with extremity bone and soft tissue tumors.

BACKGROUND AND OBJECTIVES: Limb salvage surgery remains the standard treatment in bone and soft tissue tumors. Toronto Extremity Salvage Score (TESS) is the most used quality of life measure. Our objective was to perform cross-cultural adaptation and validation in Italian, testing test-retest reliability, construct validity, and responsiveness. METHODS: We interviewed patients already treated for content validity. A total of 124 patients completed TESS and other questionnaires presurgery, at 3 months, 3 months + 2 weeks, and 6 months follow-up. We calculated intraclass correlation coefficients (ICCs) for reliability, associations with Pearson's r, and change over time with paired T tests. RESULTS: A new item regarding touch-screen devices was added to the upper extremity (UE) questionnaire. ICC resulted of 0.99 for lower extremity (LE) and 0.98 for UE patients, Pearson's r between TESS and Musculoskeletal Tumor Society was .66 and .64, EuroQol-5D-5L r was .62 and .61, and r between TESS and short form-36 physical function subscale was .76 and .71 for LE and UE groups, respectively. Paired T test results were statistically significant to detect change over time (0.03, 0.04, and 0.04 for LE groups and 0.03, 0.01, and 0.04 for UE groups). CONCLUSION: The Italian version of TESS can be used for the bone and soft tissue sarcoma population in clinical trials in Italy and with Italian speaking patients abroad to ensure patients' perspectives for efficacy and efficiency of treatments.

Decision-making in Orthopaedic Oncology: Does Cognitive Bias Affect a Virtual Patient's Choice Between Limb Salvage and Amputation?

BACKGROUND: The local treatment of extremity sarcomas usually is predicated on a decision between limb salvage and amputation. The manner in which surgical options are presented in the context of shared decision-making may influence this decision. In a population of "simulated" patients-survey respondents presented with a mock clinical vignette and then asked to choose between treatments-we assessed cognitive bias by deliberate alteration of the subjective presentation of the same objective information. QUESTIONS/PURPOSES: (1) Will the manner in which information is presented to a simulated patient, in the setting of treatment for a bone sarcoma, bias their decision regarding pursuing amputation versus limb salvage? (2) At the time of decision-making, will a simulated patient's personal background, demographics, or mood affect their ultimate decision? METHODS: Survey respondents (Amazon MTurk platform) were presented with mock clinical vignettes simulating a sarcoma diagnosis and were asked to choose between amputation and limb salvage. Specific iterations were designed to assess several described types of cognitive bias. These scenarios were distributed, using anonymous online surveys, to potential participants aged 18 years or older. Recruitment was geographically restricted to individuals in the United States. Overall, 404 respondents completed the survey. The average age of respondents was 33 years (SD 1.2 years), 60% were male and 40% were female. In all, 12% of respondents worked in healthcare. Each respondent also completed questions regarding his or her demographics and his or her current mood. Associations between the type of bias presented and the respondent's choice of limb salvage versus amputation were examined. Independent sample t-tests were used to compare means. Statistical significance was defined as p < 0.05. RESULTS: When amputation was presented as an option to mitigate functional loss (framing bias), more patients chose it than when limb salvage was presented as means for increased functional gains (23% [23 of 100] versus 10% [12 of 118], odds ratio [OR], 2.26; p = 0.010). Older simulated patients were more likely to choose limb salvage when exposed to framing bias versus younger patients (mean age 33 years versus 30 years, p = 0.02). Respondents who were employed in healthcare more commonly chose amputation versus limb salvage when exposed to framing bias (24% [eight of 35] versus 9% [17 of 183]; OR, 2.46; p = 0.02). Those who chose amputation were more likely to score higher on scales that measured depression or negative affect. CONCLUSIONS: Shared decision-making in orthopaedic oncology represents a unique circumstance in which several variables may influence a patient's decision between limb salvage and amputation. Invoking cognitive bias in simulated patients appeared to affect treatment decisions. We cannot be sure that these findings translate to the experience of actual sarcoma patients; however, we can conclude that important treatment decisions may be affected by cognitive bias and that patient characteristics (in this study, age, healthcare profession, and mood) may be associated with an individual's susceptibility to cognitive bias. We hope these observations will assist providers in the thoughtful delivery of highly charged information to patients facing difficult decisions, and promote further study of this important concept. LEVEL OF EVIDENCE: Level III, economic and decision analyses.

Quality of Life and Pain Experienced by Children and Adolescents With Cancer at Home Following Discharge From the Hospital.

An exploratory study was conducted to examine the quality of life and pain experienced by patients with pediatric cancer at home after discharge. Physical, cognitive, social, and emotional aspects of quality of life were measured and how these may be affected by age, sex, diagnosis, and pain status. The authors also characterized intensity, location, and quality of pain experienced. A sample of 33 patients participating in a larger study was selected on the basis of having pain on the day of discharge and having completed the Pediatric Quality of Life Inventory Generic, Cancer Module, Multidimensional Fatigue Scale, and the Adolescent Pediatric Pain Tool at home. Cancer diagnoses were leukemias/lymphomas (42.4%), brain/central nervous system tumors (27.3%), sarcomas (24.2%), or other (6.1%). More than half of patients reported pain (n=17; 51.5%). Patients with pain had more fatigue affecting the quality of life (P=0.01), and lower physical and emotional functioning, leading to lower overall health-related quality of life scores (P=0.011). Female individuals and adolescents reported worse emotional functioning (P=0.02 and P=0.05, respectively). Physical, cognitive, and social functioning were lowest among patients diagnosed with sarcomas (P=0.00, P=0.01, and P=0.04, respectively). It is important to understand the symptom experience of patients at home as a first step in moving towards optimal discharge teaching and treatment.

High prevalence of persistent emotional distress in desmoid tumor.

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.

Psychosocial Distress in Follow-up Care - Results of a Tablet-based Routine Screening in 202 Patients With Sarcoma.

BACKGROUND: Patients with sarcoma are particularly vulnerable to psychosocial distress. The aim of this study was to collect preliminary data on the prevalence of psychosocial distress in such patients during follow-up care and identify risk factors associated with higher psycho-oncological stress levels. PATIENTS AND METHODS: The study retrospectively enrolled 202 patients with bone or soft-tissue sarcomas who underwent routine psychosocial distress screening during their follow-up care. All patients were screened using an electronic cancer-specific questionnaire. RESULTS: Females and patients who underwent radiotherapy were more distressed. Psychosocial distress levels were markedly higher in the early postoperative phase, but approximately one-third of patients showed high psychosocial distress levels even more than 2 years postoperatively. CONCLUSION: The results underscore the importance of routine psychosocial distress screenings in patients with sarcoma, which should be performed throughout the follow-up period.

Health-related quality of life of Brazilian children and adolescents with benign and malignant solid tumours: A prospective cohort study during the first year after hospital admission.

INTRODUCTION: This study aims to assess the impact of paediatric benign and malignant solid tumours and its treatment on the health-related quality of life of children and adolescents who were followed up in a Reference Center in Pediatric Oncology in Rio de Janeiro. METHODS: It is a prospective cohort study. Quality of life assessment was performed using the PedsQL™ 4.0 Generic Core Scales and PedsQL™ 3.0 Cancer Module protocols three times: during hospital admission (T1), 6 months after admission (T2) and 1 year after admission (T3). RESULTS: We evaluated 132 patients, 59 men and 73 women, aged 2-17 years. In PedsQL™4.0, the Emotional Functioning scale was the one with the worst scores, while the scores on the Social Functioning scale was the best. In PedsQL™ 3.0, the worst domains were Procedural Anxiety and Worry. Patients with malignant bone tumours had the worst health-related quality of life. The group who received only surgery had better results. Total scores of PedsQL™4.0 and PedsQL™ 3.0 improved between T1 and T3. CONCLUSION: Children and adolescents with malignant and benign neoplasms undergo changes in quality of life as a result of the disease and treatment, but an improvement has been observed over time.

A Comparison of Limb Salvage Versus Amputation for Nonmetastatic Sarcomas Using Patient-reported Outcomes Measurement Information System Outcomes.

INTRODUCTION: The Patient-reported Outcomes Measurement Information System (PROMIS) is a scoring tool that allows comparisons between patients with rare conditions and more common ailments, or the general US population. PROMIS outcomes were compared between the limb salvage and amputee patients for nonmetastatic sarcomas to the US population. METHODS: One hundred thirty-eight patients were included in the analysis. Patients were divided into the limb salvage and amputee cohorts, as well as based on the follow-up (1 to 11 or 12+ months). RESULTS: Seven PROMIS domains were evaluated, and higher scores were found in both the limb salvage group and patients >12 months from surgery. The limb salvage group also had improved emotional health compared with the US population. DISCUSSION: Improvements in PROMIS values are observed in limb salvage patients and in patients >12 months from surgery. Limb salvage patients demonstrate improved emotional health compared with the US population.

A Mobile-Based Mindfulness and Social Support Program for Adolescents and Young Adults With Sarcoma: Development and Pilot Testing.

BACKGROUND: Approximately 70,000 adolescents and young adults (AYA) are diagnosed with cancer each year in the United States. Sarcomas carry a particularly high symptom burden and are some of the most common cancers among AYA. Recent work has documented significant levels of unmet needs among AYA with cancer, particularly the need for psychosocial support. Mobile technology may be a cost-effective and efficient way to deliver a psychosocial intervention to AYA with cancer and cancer survivors. OBJECTIVE: The two aims of this study were to (1) develop a pilot version of a mobile-based mindfulness and social support program and (2) evaluate program usage and acceptability. An exploratory aim was to examine change in psychosocial outcomes. METHODS: Thirty-seven AYA with sarcoma or sarcoma survivors, parents, and health care providers participated in the study. Semistructured interviews were conducted with 10 AYA, parents of five of the adolescents, and six health care providers. Themes from the interviews helped to inform the development of a mobile-based mindfulness pilot program and a companion Facebook-based social support group. Twenty AYA consented to participate in a single-arm pre-post evaluation of the program; 17 downloaded the app and joined the Facebook group. Seven of these participants had participated in the semistructured interviews. Six additional health care providers consented to participate in the evaluation stage. RESULTS: On average, participants completed 16.9 of the 28 unique sessions and used the mindfulness app for a mean 10.2 (SD 8.2) days during the 28-day evaluation period. The majority of participants (16/17) engaged in the social group and posted at least one reply to the moderator's prompts. The mean number of responses per person to the moderator of the social group was 15.2 of 31 (49%, range 0%-97%). Both AYA and health care providers responded positively to the Mindfulness for Resilience in Illness program and offered useful recommendations for improvements. Exploratory psychosocial analyses indicated there were no significant differences from pretest to posttest on measures of perceived social support, mindfulness, body image, or psychological functioning. CONCLUSIONS: This study offers preliminary support for the feasibility and acceptability of a mobile-based mindfulness and Facebook-based social support program for AYA with sarcoma. The feedback from AYA and health care providers will assist in creating a fully developed intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT03130751; https://clinicaltrials.gov/ct2/show/NCT03130751.

Symptom Burden and End-of-Life Palliative Treatments during the Last Two Weeks of Life in Patients with Advanced Musculoskeletal Sarcoma.

Background: Musculoskeletal sarcomas (MSSs) are rare cancers and often aggressive tumors that originate from mesenchymal tissue. Patients with advanced MSS often report difficulties with symptom burden, which can reduce their health-related quality-of-life. Objective: The aim of this study was to describe the patterns of the physical symptoms of MSS patients in the palliative setting and to detail the palliative treatment used in the last two weeks of life. Design: Retrospective study using the electronic patient records from a single institution. Setting/Subjects: A retrospective study was carried out in a sample of 46 consecutive MSS patients with locally advanced/metastatic disease, who were hospitalized and died in our department. The median age of these patients was 56 years at death. Measurements: Symptom burden and medical intervention during the last two weeks of life were collected. Results: The most frequent physical symptoms were pain and dyspnea in 93% and 78% of patients, respectively, while only 17% of patients suffered from nausea. A total of 98% of patients required opioids, and most patients were treated with morphine through either subcutaneous or intravenous continuous injection. Nonsteroidal anti-inflammatory drugs and acetaminophen were administered to 79% of patients. Corticosteroids were administered for the relief of dyspnea to 83% of patients. Of the patients, 46% received palliative chemotherapy within the last two weeks of life, and the oral treatment was continued until a median of 5.6 days before death. In addition, 39% of patients received a sedative treatment during the last two weeks of life for uncontrolled refractory symptoms. Conclusions: The symptom burden experienced by advanced MSS patients is profound at the end of life for all palliative approaches. Therefore, palliative medicine is an important and even crucial component of the continuum of care, allowing for aggressive symptom management with a variety of medical interventions, including palliative sedation.

Health-related quality of life, psychosocial functioning, and unmet health needs in patients with sarcoma: A systematic review.

OBJECTIVE: Health-related quality of life (HRQoL), psychosocial distress, and unmet health needs are important patient-reported outcomes (PROs) for patients with sarcoma treated with curative intent. Syntheses of data on these PROs in patients with sarcoma are limited. METHODS: A systematic review of peer-reviewed literature published between 2007 and 2017 was conducted using five databases, guided by the PRISMA and Cochrane reporting guidelines. RESULTS: Of 7240 articles identified, 31 were included in this review. Compared with healthy individuals, patients with sarcoma frequently scored lower in physical and psychological HRQoL domains and experienced higher rates of self-image issues, depression, and suicide. However, outcomes for patients with sarcoma were relatively comparable to those with other malignancies. Anxiety symptoms were more common in the diagnosis phase, while depressive symptoms were more common in the treatment phase. Patients who are older, female, and socially isolated often reported lower HRQoL. As a sarcoma-specific HRQoL instrument is not available, and all studies have used generic instruments, key issues may have been omitted. CONCLUSION: There are few studies reporting data for HRQoL, psychosocial issues, or unmet health needs in this population. A sarcoma-specific HRQoL instrument is needed to accurately describe outcomes in this population. There is a need for further qualitative research to conceptualise the HRQoL issues relevant to this population, against which to map existing HRQoL instruments and guide adaptation or creation of a new instrument.

'When something is this rare how do you know bad really is bad ?'-views on prognostic discussions from patients with advanced soft tissue sarcoma.

OBJECTIVES: Prognostic disclosure among patients with cancer permits open informed discussion about treatment preferences and encourages advance care planning. In rare cancers such as soft tissue sarcoma, discussions regarding prognostication are challenging. Little is known about the consequences of this for patients or their preferences for such information. This qualitative study explores patient-centered accounts of the value and timing of prognostic discussions. METHODS: 24 semistructured interviews were conducted with soft tissue sarcoma patients attending one London cancer centre: 66% female, median age 53 (range 19-82). The study was cross-sectional and participants were at different stages of the advanced disease trajectory. Interviews were digitally recorded, transcribed verbatim and analysed thematically using the framework approach. RESULTS: All participants understood the incurable nature of advanced sarcoma. However, prognostic discussions were rare, always patient initiated and did not include known survival data, despite direct participant enquiry. Most participants did not wish to discuss prognosis at initial diagnosis but wished to be offered the opportunity to discuss this at intervals of disease progression, despite reservations it may not be helpful. Participants expected discussions to be clinician initiated. Three themes emerged to explain this position and included (1) Rarity causing prognostic uncertainty referring to patient belief that prognostication in rare cancers was less likely to be accurate than for common tumours; (2) Avoiding the negatives referring to a wish not to hear unfavourable information and (3) Physical symptoms a better prognostic indicator than 'physician guess'. CONCLUSIONS: Although 17/24 participants preferred not to discuss prognosis at initial diagnosis, they wished to have the opportunity to revisit prognostic discussions at intervals of disease progression. This may facilitate better advance care planning and end of life care.