Risk factors for neurocognitive impairment, emotional distress, and poor quality of life in survivors of pediatric rhabdomyosarcoma: A report from the Childhood Cancer Survivor Study.

BACKGROUND: Prevalence and risk of poor psychological outcomes following rhabdomyosarcoma (RMS) are not well-established. METHODS: Participants in this cross-sectional, case-control study (n = 713 survivors, 42.5% female; mean [SD] age, 30.5 [6.6] years; n = 706 siblings, 57.2% female; mean age, 32.8,[7.9] years) completed measures of neurocognition, emotional distress, and health-related quality of life (HRQOL). Multivariable logistic regression models identified treatments, health behaviors, and chronic conditions associated with impairment. RESULTS: Relative to siblings, more survivors reported neurocognitive impairment (task efficiency: 21.1% vs. 13.7%, emotional regulation: 16.7% vs. 11.0%, memory: 19.3% vs. 15.1%), elevated emotional distress (somatic distress: 12.9% vs. 4.7%, anxiety: 11.7% vs. 5.9%, depression: 22.8% vs. 16.9%) and poorer HRQOL (physical functioning: 11.1% vs. 2.8%, role functioning due to physical problems: 16.8% vs. 8.2%, pain: 17.5% vs. 10.0%, vitality: 22.3% vs. 13.8%, social functioning: 14.4% vs. 6.8%, emotional functioning: 17.1% vs. 10.6%). Cranial radiation increased risk for impaired task efficiency (odds ratio [OR], 2.30; 95% confidence interval [CI], 1.14-4.63), whereas chest and pelvic radiation predicted increased risk of physical functioning (OR, 2.68; 95% CI, 1.16-6.21 and OR, 3.44; 95% CI, 1.70-6.95, respectively). Smoking was associated with impaired task efficiency (OR, 2.06; 95% CI, 1.14-3.70), memory (OR, 2.23; 95% CI, 1.26-3.95), anxiety (OR, 2.71; 95% CI, 1.36-5.41) and depression (OR, 1.77; 95% CI, 1.01-3.11). Neurologic conditions increased risk of anxiety (OR, 2.30; 95% CI, 1.04-5.10), and hearing conditions increased risk of depression (OR, 1.79; 95% CI, 1.05-3.03). Neurologic and hearing conditions, respectively, were associated with impaired memory (OR, 2.44; 95% CI, 1.20-4.95 and OR, 1.87; 95% CI, 1.05-3.35) and poor health perception (OR, 2.62; 95% CI, 1.62-1.28 and OR, 2.33; 95% CI, 1.34-4.06). CONCLUSIONS: RMS survivors are at significant risk for poor psychological outcomes. Advancing therapies for local control, smoking cessation, and managing chronic medical conditions may mitigate poor outcomes following RMS.

Psychosocial well-being of long-term survivors of pediatric head-neck rhabdomyosarcoma.

BACKGROUND: Head and neck rhabdomyosarcoma (HNRMS) survivors are at risk to develop adverse events (AEs). The impact of these AEs on psychosocial well-being is unclear. We aimed to assess psychosocial well-being of HNRMS survivors and examine whether psychosocial outcomes were associated with burden of therapy. PROCEDURE: Sixty-five HNRMS survivors (median follow-up: 11.5 years), treated in the Netherlands and the United Kingdom between 1990 and 2010 and alive ≥2 years after treatment visited the outpatient multidisciplinary follow-up clinic once, in which AEs were scored based on a predefined list according to the Common Terminology Criteria for Adverse Events. Survivors were asked to complete questionnaires on health-related quality of life (HRQoL; PedsQL and YQOL-FD), self-perception (KIDSCREEN), and satisfaction with appearances (SWA). HRQoL and self-perception scores were compared with reference values, and the correlation between physician-assessed AEs and psychosocial well-being was assessed. RESULTS: HNRMS survivors showed significantly lower scores on PedsQL school/work domain (P ≤ 0.01, P = 0.02, respectively), YQOL-FD domains negative self-image and positive consequences (P ≤ 0.01, P = 0.04, respectively) compared with norm data; scores on negative consequences domain were significantly higher (P = 0.03). Over 50% of survivors negatively rated their appearances on three or more items. Burden of AEs was not associated with generic HRQoL and self-perception scores, but was associated with disease-specific QoL (YQOL-FD). CONCLUSION: In general, HRQoL in HNRMS survivors was comparable to reference groups; however, survivors did report disease-specific consequences. We therefore recommend including specific questionnaires related to difficulties with facial appearance in a systematic monitoring program to determine the necessity for tailored care.

Tumour control and Quality of Life in children with rhabdomyosarcoma treated with pencil beam scanning proton therapy.

PURPOSE: To assess clinical outcomes in children with rhabdomyosarcoma (RMS) treated with pencil beam scanning (PBS) proton therapy (PT). METHODS AND MATERIALS: Eighty-three RMS (embryonal, n=74; 89%) patients treated between January 2000 and December 2014 were included. The median age was 4.5years (range, 0.8-15.5). All patients received systemic chemotherapy according to prospective protocols. Patients had low-, intermediate-, and high-risk disease in 24%, 63%, and 13% of cases, respectively. The median total dose delivered was 54Gy(RBE) (range, 41.4-64.8). RESULTS: After a median follow-up time of 55.5 months (range, 0.9-126.3), local failure occurred in 16 patients. The 5-year local-control survival rate was 78.5% [95% confidence interval (CI), 69.5-88.5%]. Significant predictors for local failure were group/stage, tumour location, and size. Fourteen patients (16%) died, all from tumour progression. The 5-year overall survival was 80.6% (95%CI, 71.8-90.0%). The 5-year incidence of grade 3 non-ocular late toxicity was 3.6% (95%CI, 1-12%). No grade 4-5 late toxicities were observed. One radiation-induced malignancy was observed (1.2%). The Quality of Life (QoL) scores increased significantly after PT compared to baseline values. CONCLUSIONS: PBS PT led to excellent outcome in children with RMS. Late non-ocular toxicity was minimal and QoL good.

Using play therapy in paediatric palliative care: listening to the story and caring for the body.

To be truly comprehensive, palliative care for children must address more than pain control and symptom management. Holistic care also encompasses attention to the child's relationships, hopes, fears and wishes. Parents and caregivers of dying children are generally the primary decision-makers in the child's care and can find the transition from active, to palliative care, particularly difficult. Nurses who understand the parents' perspective can better support them. Children reveal their hopes and fears through play. By being attuned to symbols and themes in play, nurses can better interpret the dying child's journey. Nurses can facilitate communication and connection between parents and child and thereby promote healing during the dying process.

Physical impairment and social adaptation in adult survivors of childhood and adolescent rhabdomyosarcoma: A report from the Childhood Cancer Survivors Study.

BACKGROUND: Despite interest in the well-being of adult survivors of childhood rhabdomyosarcoma (RMS), few studies have examined their health-related quality of life (HRQOL). This study evaluated physical and social aspects of HRQOL among long-term childhood RMS survivors relative to a sibling comparison group, and assessed whether physical impairment among RMS survivors adversely affected their ability to achieve adult life goals. METHODS: Using baseline data from the Childhood Cancer Survivor Study, we evaluated self-reported physical impairment and social adaptation among 417 survivors of childhood RMS and 2685 siblings > or =18 years of age at survey completion. RESULTS: Survivors were more likely than siblings to report physical impairment, characterized by: at least one medically diagnosed condition, limitations in the performance of routine activities, a health-related inability to work or attend school, cancer-related pain. Survivors were less likely than siblings to have completed high school, ever worked a job, or ever been married. The odds of completing high school were lower among survivors with performance limitations, a health-related inability to work or attend school, or moderate to high levels of cancer-related pain. Survivors who reported cancer-related pain had an increased likelihood of ever being married. CONCLUSIONS: The majority of adult survivors of pediatric RMS are successful in attaining adult life goals despite higher reported occurrence of physical impairment than their sibling counterparts. Additional studies are needed to advance our understanding of other aspects of HRQOL in this population of pediatric cancer survivors.

Long-term medical effects of childhood and adolescent rhabdomyosarcoma: a report from the childhood cancer survivor study.

BACKGROUND: This study was conducted to evaluate the incidence of adverse medical conditions and to assess the risk of developing these conditions in a cohort of long-term survivors of rhabdomyosarcoma (RMS) diagnosed before age 21. PROCEDURE: Using data from the Childhood Cancer Survivor Study (CCSS), we evaluated the incidence of self-reported adverse medical conditions for 606 RMS survivors and 3,701 siblings of cancer survivors. Cancer and treatment data abstracted from medical records were used to evaluate the effects of primary tumor site and combined modality therapy on the risk of developing sequelae in survivors. RESULTS: The relative risk (RR) for developing sequelae among survivors compared with siblings was greatest within 5 years after diagnosis. RR was elevated more than 5 years after diagnosis for several conditions (RR, 95% CI) as follows: eye impairment (cataract: 7.4, 2.9-18.9; visual disturbances: 3.2, 2.0-5.1; very dry eyes: 2.0, 1.2-3.3), endocrine impairment (growth hormone deficiency: 83.9, 33.0-213.6; hypothyroidism: 6.9, 4.1-11.3; need for medications to induce puberty: 90.4, 30.2-270.5), cardiopulmonary impairment (congestive heart failure: 43.0, 12.7-145.5; angina-like symptoms: 2.0, 1.3-2.9), neurosensory impairment (legal blindness: 9.8, 4.8-20.0; abnormal sensations: 1.5, 1.0-2.2), and neuromotor impairment (repeated seizures: 2.3, 1.2-4.4; motor problems: 3.7, 2.2-6.4; problems chewing or swallowing: 3.8, 1.9-7.5). CONCLUSIONS: Survivors are at risk for developing sequelae many years after their initial diagnosis and treatment. Continued medical surveillance is necessary to ensure the long-term health and well-being of RMS survivors.

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.

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.

[How do parents adjust to a child with cancer? A clinical study of the psychosocial status of parents of children with cancer]. / Wie leben Eltern mit einem Krebskranken Kind? Eine klinische Untersuchung zur psychosozialen Situation der Eltern krebskranker Kinder.

As chronic life-threatening diseases, cancer or leukemia in children generates an extreme situation for the family involved that can go on for years. Psychosocial care for all family members by a pediatric oncologist should be integrated into the treatment plan, as this is both helpful and necessary.

Social adjustment of children successfully treated for cancer.

Compared 56 children between the ages of 5 and 12 years to a sample of healthy controls. All cancer patients had been treated between the ages of 2 and 5 years. Children completed the Self-Perception Profile for children and questionnaires assessing social adjustment. Parent and teacher ratings were also obtained. The cancer patients reported greater feelings of isolation than the controls but otherwise few differences were noted. There were few differences between cancer patients and controls in the teacher and parent report. Results are discussed in terms of the need to investigate behavioral sequelae of childhood illness and directions for future research.

Psychosocial ramifications of childhood malignancy for the child and family.

The changes that occur in a child's life following the diagnosis of cancer extend to other members of the family and, through time, far beyond the biologic cure of the disease. The responses of the family to this stress and the ways in which they cope with it will depend partially on their own resources and partially on the severity of the disease and its treatment. This paper describes the factors that influence the family's adaptation to illness and presents a case example that illustrates criteria useful in assessing the need for psychologic consultation in childhood cancer patients and their families. Also presented are guidelines for the physician in his/her continuing observation and follow-up of these patients and families.

Immediate and long-term posttherapy neuropsychologic performance in children with acute lymphoblastic leukemia treated without central nervous system radiation.

Intellectual and neuropsychologic test performance was studied in 41 children with acute lymphoblastic leukemia within one year of completing multidrug therapy in which intrathecal methotrexate was the sole agent of central nervous system prophylaxis. To control for possible effects of intensive systemic chemotherapy and the disruptive psychosocial effects of treatment on psychometric test function, 33 children with embryonal rhabdomyosarcoma who had no CNS disease or treatment were similarly studied. All patients studied had been in continuous complete remission since diagnosis. Within each diagnostic group, patients were placed in a younger (less than 8 years) or an older (more than 8 years) group depending on age at the time of testing. No significant difference was found in intellectual function between the young ALL and ERMS groups or between the total ALL and ERMS groups. Further five-year follow-up of 12 of the youngest ALL patients showed no significant long-term change in intellectual or other specific neuropsychologic functions. Children with ALL who remained in continuous complete remission from diagnosis and who were treated with prophylactic intrathecal methotrexate and no CNS radiation did not have global or specific neuropsychologic impairment.