PurposeAdolescent and young adult cancer survivors (AYAs) are at increased risk of long-term and late effects, and experience unmet needs, impacting their health-related quality of life (HRQoL). In... Show morePurposeAdolescent and young adult cancer survivors (AYAs) are at increased risk of long-term and late effects, and experience unmet needs, impacting their health-related quality of life (HRQoL). In order to provide and optimize supportive care and targeted interventions for this unique population, it is important to study HRQoL factors' interconnectedness on a population level. Therefore, this network analysis was performed with the aim to explore the interconnectedness between HRQoL factors, in the analysis described as nodes, among long-term AYAs.MethodsThis population-based cohort study used cross-sectional survey data of long-term AYAs, who were identified by the Netherlands Cancer Registry (NCR). Participants completed a one-time survey (SURVAYA study), including the EORTC survivorship questionnaire (QLQ-SURV111) to assess their long-term HRQoL outcomes and sociodemographic characteristics. The NCR provided the clinical data. Descriptive statistics and a network analysis, including network clustering, were performed.ResultsIn total, 3596 AYAs (on average 12.4 years post diagnosis) were included in our network analysis. The network was proven stable and reliable and, in total, four clusters were identified, including a worriment, daily functioning, psychological, and sexual cluster. Negative health outlook, part of the worriment cluster, was the node with the highest strength and its partial correlation with health distress was significantly different from all other partial correlations.ConclusionThis study shows the results of a stable and reliable network analysis based on HRQoL data of long-term AYAs, and identified nodes, correlations, and clusters that could be intervened on to improve the HRQoL outcomes of AYAs. Show less
Background: Sarcomas account for almost 11% of all cancers in adolescents and young adults (AYAs; 18-39 years). AYAs are increasingly recognized as a distinct oncological age group with its own... Show moreBackground: Sarcomas account for almost 11% of all cancers in adolescents and young adults (AYAs; 18-39 years). AYAs are increasingly recognized as a distinct oncological age group with its own psychosocial challenges and biological characteristics. Social functioning has been shown to be one of the most severely affected domains of health-related quality of life in AYA cancer survivors. This study aims to identify AYA sarcoma survivors with impaired social functioning (ISF) and determine clinical and psychosocial factors associated with ISF. Methods: AYAs from the population-based cross-sectional sarcoma survivorship study (SURVSARC) were included (n = 176). ISF was determined according to the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 social functioning scale, and age- and sex-matched norm data were used as reference. Results: The median time since diagnosis was 6.2 years (range, 1.8-11.2). More than one-quarter (28%) of AYA sarcoma survivors experienced ISF. Older age, higher tumor stage, comorbidities, lower experienced social support, uncertainty in relationships, feeling less attractive, sexual inactivity, unemployment, and financial difficulties were associated with ISF. In a multivariable analysis, unemployment (OR, 3.719; 95% CI, 1.261-10.967) and having to make lifestyle changes because of financial problems caused by one's physical condition or medical treatment (OR, 3.394; 95% CI, 1.118-10.300) were associated with ISF; better experienced social support was associated with non-ISF (OR, 0.739; 95% CI, 0.570-0.957). Conclusion: More than one-quarter of AYA sarcoma survivors experience ISF long after diagnosis. These results emphasize the importance of follow-up care that is not only disease-oriented but also focuses on the psychological and social domains. Plain Language Summary Sarcomas account for almost 11% of all cancers in adolescents and young adults (AYAs; 18-39 years). The AYA group is increasingly recognized as a distinct oncological age group with its own psychosocial challenges and biological characteristics.Social functioning has been shown to be severely affected in AYA cancer survivors.A population-based questionnaire study to identify AYA sarcoma survivors with impaired social functioning (ISF) and determine factors associated with ISF was conducted. More than one-quarter of AYA sarcoma survivors experience ISF long after diagnosis. These results emphasize the importance of follow-up care that is not only disease-orientated but also focuses on the psychological and social domains. Show less
Pellegrini, I.; Drabbe, C.; Gruenhagen, D.J.; Sande, M.A.J. van de; Haan, J.J. de; Keymeulen, K.B.M.I.; ... ; Husson, O. 2022
Simple Summary: Fear of cancer recurrence is often reported as an unmet concern by cancer patients, and, to our knowledge, it has not been assessed yet in sarcoma survivors. We conducted a... Show moreSimple Summary: Fear of cancer recurrence is often reported as an unmet concern by cancer patients, and, to our knowledge, it has not been assessed yet in sarcoma survivors. We conducted a population-based cross sectional questionnaire study to assess the prevalence and characteristics of fear of cancer recurrence amongst sarcoma survivors. We demonstrated that severe fear of cancer recurrence is common in sarcoma survivors and that high levels are associated with decreased global health status. Fear of cancer recurrence deserves more attention in optimal sarcoma survivorship care. To guarantee adequate patient care, the collaboration between health care professionals should be encouraged, and structured support programs should be developed to deliver interventions in a correct and time adequate environment. Fear of cancer recurrence (FCR) is often reported as an unmet concern by cancer patients. The aim of our study was to investigate (1) the prevalence of FCR in sarcoma survivors; (2) the factors associated with a higher level of FCR; the relationship between (3) FCR and global health status and (4) FCR and use of follow-up care. Methods: A cross-sectional study was conducted among sarcoma survivors 2 to 10 years after diagnosis. Patients completed the Cancer Worry Scale (CWS), the global health status subscale of the EORTC QLQ-C30 and a custom-made questionnaire on follow-up care. Results: In total, 1047 patients were included (response rate 55%). The prevalence of high FCR was 45%. Factors associated with high FCR were female sex with 1.6 higher odds (95% CI 1.22-2.25; p = 0.001); having >= 1 comorbidities and receiving any treatment other than surgery alone with 1.5 (95% CI 1.07-2.05; p = 0.017) and 1.4 (95% CI 1.06-1.98; p = 0.020) higher odds, respectively. Patients on active follow-up had 1.7 higher odds (95% CI 1.20-2.61; p = 0.004) and patients with higher levels of FCR scored lower on the global health status scale (72 vs. 83 p <= 0.001). Conclusions: Severe FCR is common in sarcoma survivors and high levels are related to a decreased global health status. FCR deserves more attention in sarcoma survivorship, and structured support programs should be developed to deliver interventions in a correct and time adequate environment. Show less
Drabbe, C.; Grunhagen, D.J.; Houdt, W.J. van; Braam, P.M.; Soomers, V.L.M.N.; Hage, J.A. van der; ... ; Graaf, W.T.A. van der 2021
Background: Health-related quality of life (HRQoL) data of sarcoma survivors are scarce and the impact of age remains unclear. The aims of this population-based study were to (i) compare HRQoL... Show moreBackground: Health-related quality of life (HRQoL) data of sarcoma survivors are scarce and the impact of age remains unclear. The aims of this population-based study were to (i) compare HRQoL scores amongst three age-groups [adolescents and young adults (AYA, aged 18-39 years), older adults (OA, aged 40-69 years) and elderly (aged >= 70 years)]; (ii) compare HRQoL of each sarcoma survivor age group with an age- and sex-matched normative population sample; (iii) determine factors associated with low HRQoL per age group.Methods: Dutch sarcoma survivors, who were 2-10 years after diagnosis, were invited to complete the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30-questions questionnaire on HRQoL.Results: In total, 1099 survivors (58% response rate) completed the questionnaire: 186 AYAs, 748 OAs and 165 elderly. The median time since diagnosis for all patients was 5.2 years. Bone sarcomas were seen in 41% of AYAs, 22% of OAs and in 16% of elderly survivors (P < 0.01). AYA and OA survivors reported statistically significant and clinically meaningful worse physical, role, cognitive, emotional and social functioning compared with a matched norm population, which was not the case for elderly survivors. AYAs reported significantly worse scores on emotional and cognitive functioning compared with OA and elderly survivors. Malignant peripheral nerve sheath tumour, osteosarcoma and chordoma were the subtypes of which survivors reported the lowest HRQoL scores in comparison with the norm. For all age groups, chemotherapy, having a bone sarcoma and having comorbidities were most frequently associated with low scores on HRQoL subscales, whereas a shorter time since diagnosis was not.Conclusion: In this nationwide sarcoma survivorship study, the disease and its treatment had relatively more impact on the HRQoL. of AYA and OA survivors than on elderly survivors. These results emphasise the need for personalised followup care that not only includes risk-adjusted care related to disease relapse, but also age-adjusted care. Show less
IJzerman, N.S.; Drabbe, C.; Hollander, D. den; Mohammadi, M.; H. van boven; Desar, I.M.E.; ... ; Graaf, W.T.A. van der 2020
Gastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by KIT/PDGFR mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and... Show moreGastrointestinal stromal tumour (GIST) is a disease of older adults and is dominated by KIT/PDGFR mutations. In children, GIST is rare, predominantly occurs in girls, has a stomach location and generally lacks KIT/PDGFR mutations. For young adults (YA), aged 18 to 40 years, the typical phenotypic and genotypic patterns are unknown. We therefore aimed to describe the clinical, pathological and molecular characteristics of GIST in in YA. YA GIST patients registered in the Dutch GIST Registry (DGR) were included, and data were compared to those of older adults (OA). From 1010 patients in the DGR, 52 patients were YA (54% male). Main tumour locations were stomach (46%) and small intestine (46%). GIST genetic profiles were mutations in KIT (69%), PDGFRA (6%), SDH deficient (8%), NF1 associated (4%), ETV6-NTRK3 gene fusion (2%) or wildtype (10%). Statistically significant differences were found between the OA and YA patients (localisation, syndromic and mutational status). YA presented more often than OA in an emergency setting (18% vs. 9%). The overall five-year survival rate was 85%. In conclusion, YA GISTs are not similar to typical adult GISTs and also differ from paediatric GISTs, as described in the literature. In this series, we found a relatively high percentage of small intestine GIST, emergency presentation, 25% non-KIT/PDGFRA mutations and a relatively good survival. Show less
