Long-term functional outcomes and quality of life in adult survivors of childhood extremity sarcomas: a report from the St. Jude Lifetime Cohort Study

Abstract

PURPOSE This study compared measured physical performance, health-related-quality-of-life (HRQOL), and social role attainment between extremity sarcoma survivors and controls, and evaluated associations between disease and treatment exposures, health conditions, and performance measures. METHODS Survivors of extremity sarcoma from the St. Jude Lifetime cohort, and controls frequency matched by age-, sex-, and race completed physical performance testing and questionnaires. Survivors with Z-scores on outcome measures ≤ −2.0 SD (compared to controls) were categorized with severe impairment/limitation. RESULTS Among 206 survivors (52.4% male median age 36 (range 19–65) years, 37% had low relative lean mass, 9.7% an ejection fraction <50%, 51.5% diffusion capacity for carbon monoxide <75%, 27.7% sensory and 25.2% motor neuropathy, and 78.2% musculoskeletal complications. Severe impairments/limitations were present among ≥ 25% of survivors on fitness, balance, and physical HRQOL measures, and among ≥ 15% on strength and activity of daily living measures. Lower extremity tumor location (OR 8.23, 95% CI 2.54–26.67, P-value 0.0004) and amputation (OR 8.07, 95% CI 3.06–21.27, P-value <0.0001) were associated with poor fitness. Poor fitness was associated with increased odds of scoring <40 on the SF-36 physical component summary (OR 4.83, 95% CI 1.95–11.99, P-value 0.001) and role-physical subscale (OR 3.34, 95% CI 1.33–8.43, P-value 0.01). Survivors and controls had similar rates of marriage, independent living, employment and college attendance. CONCLUSIONS Extremity sarcoma survivors experience high rates of physical impairment, and report lower than expected physical HRQOL. However, they are as likely as peers to be married, live independently, be employed, and attend college. IMPLICATIONS FOR SURVIVORS Follow-up for extremity sarcoma survivors should include assessment of need for further orthopedic care and rehabilitation to address cardiopulmonary and musculoskeletal health.

Olaratumab Exerts Antitumor Activity in Preclinical Models of Pediatric Bone and Soft Tissue Tumors through Inhibition of Platelet-Derived Growth Factor Receptor α

“…Currently, no standard of care exists for this patient population, and overall survival remains poor (14)(15)(16). For pediatric cancer survivors, the possibility of debilitating longterm side effects, chronic health conditions, and even secondary cancers resulting from demanding therapeutic regimens remains (17,18). Therefore, it is of the utmost importance to identify and evaluate targeted agents in the pediatric setting to improve patient outcome.…”

The Role of Exercise in Pediatric and Adolescent Cancers: A Review of Assessments and Suggestions for Clinical Implementation

“…Quality of life in both childhood and adult cancer patients has been well researched and there are a number of validated age-specific scales designed to assess the emotional, physical, and social aspects of one's life [21,45,53,[56][57][58]. As part of a comprehensive rehabilitation plan for CCS, the authors support the importance of QoL assessment as part of the rehabilitation plan.…”

“…Chemotherapy for pediatric cancer suppresses the immune system and may interfere with normal growth, increasing susceptibility to infection and stunting or delaying musculoskeletal development during treatment [49]. Treatments may interfere with physiological growth and development in children and adolescents and have an important impact on health status later in life, while some late toxicities (e.g., pulmonary fibrosis, obesity, hypertension, cardiovascular disease) may cause premature death [11,14,16,17,20,21,25,28,31]. It has been estimated that 62% of adult survivors of childhood malignancy have ≥1, and 38% ≥2, treatment-induced chronic health conditions, and 28% a severe or life-threatening problem such as cardiomyopathy [30].…”

“…It is clear that psychological or social consequences interfere with quality of life (QoL), which comprises elements of physical, functional, social, and psychological health [21,22,47,[55][56][57][58]. QoL may be affected by the level of integration into society, as measured by the survivors' probability (compared to age and sex-matched general population peers) of securing employment or health insurance, or of marrying [57].…”

“…As a result, adult survivors of childhood cancer may suffer from cardiomyopathy, heart valve and conduction disorders [14,15], increased cardiovascular risk factors including hypertension, dyslipidemia [16][17][18], and obesity [19,20], pulmonary disorders [21,22], endocrine disorders [23] including hypothalamic-pituitary-adrenal disorders [24], diabetes mellitus [25], ovarian and Leydig cell dysfunction [26], and hypothyroidism [27]. Survivors of childhood cancer are also at increased risk for neurocognitive and neurosensory impairment including ocular degeneration, hearing loss and neuropathy [28][29][30][31], metabolic disturbances (abnormal blood counts, liver and kidney dysfunction, and osteoporosis) [28][29][30]32,33], transfusion-associated infections [30], and increased risk of subsequent neoplasm [14].…”