Clinical trials in desmoid-type fibromatosis in children and adults:A systematic review

Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm, which occurs in children and adults, with a peak incidence in young adults. For the majority of the patients, DTF is a chronic and symptomatic disease, which affects health-related quality of life. Systemic treatment regimens tend to differ for patients treated by pediatric oncologists compared to medical oncologists. This systematic review identified 14 clinical trials in children and adults with DTF. Tumor response and progression-free survival rates varied widely between studies and study populations. Treatment choices for patients with DTF are based on a paucity of (randomized) trials. Treatment principles of DTF are similar in pediatric and adult oncology, but the treatment itself is different. This seems mostly driven by a lack of tyrosine kinase inhibitor (TKI) accessibility in pediatric oncology. An insufficient number of studies examined patient-reported outcomes, which are extremely important for patients with a chronic disease like DTF.</p

The views of cancer patients of Turkish, Moroccan, Surinamese, and Dutch-Caribbean descent on diagnosis, treatment and prognosis:A systematic literature review

Background: The number of international migrants is increasing worldwide. The four major non-western ethnic groups in the Netherlands are Turkish, Moroccan, Surinamese, and Dutch-Caribbean. This review examined the scientific literature on the views of cancer patients from these four ethnic groups on cancer diagnosis, treatment, and prognosis. Methods: A systematic literature review was conducted using the databases EMBASE, Medline Web of Science, and Cochrane Central Register. Studies with patients who were of Turkish, Moroccan, Surinamese, and Dutch-Caribbean descent were included. Both qualitative and quantitative studies were included, and thematic analysis was performed. The methodological quality was assessed using the Mixed Methods Appraisal Tool. Results: Thirteen studies were conducted in Turkey on Turkish cancer patients, while three were conducted in the Netherlands on Turkish and Moroccan cancer patients. Four themes emerged from the included studies: disclosure of diagnosis, communication, information provision, and decision-making. The majority of cancer patients in Turkey wanted information regarding their diagnosis and treatment. However, disclosure of a cancer diagnosis was rarely discussed with cancer patients in Turkey, whereas in the Netherlands it was provided directly. Family members in both the host and native countries had a strong influence on communication and decision-making. No literature on this topic for Surinamese or Dutch-Caribbean cancer patients was found. Conclusion: Although major ethnic groups live in host countries, there is a lack of knowledge on optimal communication and information disclosure on cancer to patients and their families. Policy summary: Further research into the views of ethnic groups on how to communicate about cancer is essential to ensuring that every patient receives optimal care and treatment.</p

Same Journey, Different Paths:Caregiver Burden among Informal Caregivers of Adolescent and Young Adult Patients with an Uncertain or Poor Cancer Prognosis (UPCP)

A minority of adolescent and young adult cancer patients (AYA) live with an uncertain or poor prognosis (UPCP). Caring for a young, advanced cancer patient can lead to caregiver burden. This study aims to provide insight into burden on informal caregivers of AYA cancer patients with UPCP. In-depth, semistructured interviews were conducted with parents (n = 12), siblings (n = 7), friends (n = 7), and partners (n = 13). Thematic analysis was performed to derive themes from the data. Participants reported sleeping problems and stress. They struggle with uncertainty, fear, loss, and negative emotions. Family life is altered due to solely taking care of the children, but also the AYA. Contact with friends and family is changed. The relationship to the AYA can shift positively (e.g., becoming closer) or negatively (e.g., more conflict or no longer being attracted). Participants were under pressure, having to take on many responsibilities and multiple roles. In the financial domain, they report less income and often must continue working. A high amount of caregiver burden is experienced among informal caregivers of AYAs with UPCP. Yet only part of the impact appears to be age specific. Specific, age-adjusted interventions can be developed to lower the burden.</p

Same Journey, Different Paths:Caregiver Burden among Informal Caregivers of Adolescent and Young Adult Patients with an Uncertain or Poor Cancer Prognosis (UPCP)

A minority of adolescent and young adult cancer patients (AYA) live with an uncertain or poor prognosis (UPCP). Caring for a young, advanced cancer patient can lead to caregiver burden. This study aims to provide insight into burden on informal caregivers of AYA cancer patients with UPCP. In-depth, semistructured interviews were conducted with parents (n = 12), siblings (n = 7), friends (n = 7), and partners (n = 13). Thematic analysis was performed to derive themes from the data. Participants reported sleeping problems and stress. They struggle with uncertainty, fear, loss, and negative emotions. Family life is altered due to solely taking care of the children, but also the AYA. Contact with friends and family is changed. The relationship to the AYA can shift positively (e.g., becoming closer) or negatively (e.g., more conflict or no longer being attracted). Participants were under pressure, having to take on many responsibilities and multiple roles. In the financial domain, they report less income and often must continue working. A high amount of caregiver burden is experienced among informal caregivers of AYAs with UPCP. Yet only part of the impact appears to be age specific. Specific, age-adjusted interventions can be developed to lower the burden.</p

The prolonged diagnostic pathway of young adults (Aged 25–39) with cancer in the United Kingdom:Results from the young adult cancer patient journey study

Purpose: Teenagers and young adults (TYAs; aged 13–24) experience prolonged intervals to cancer diagnosis. Insight into diagnostic intervals in young adults (YAs; aged 25–39) and sub-groups at risk for long intervals is lacking. We investigated the diagnostic pathway of YA cancer patients, examined patient and tumor characteristics associated with its length, and compared the patient interval length of our sample with a TYA cohort. Methods: In this cross-sectional survey YAs diagnosed with cancer in the UK in the past five years completed a questionnaire describing their patient (time from first symptom to first doctor consultation) and healthcare interval (from first consultation until consultation with a cancer specialist), sociodemographic, and clinical characteristics. Associations between characteristics and interval length were examined and compared with previously published data in TYAs. Results: Among 341 YAs the patient interval lasted ≥2 weeks, ≥1 month, and ≥3 months in 60%, 42%, and 21%, respectively, compared to 48%, 27%, and 12% in the TYA group. The healthcare interval lasted ≥2 weeks, ≥1 month, and ≥3 months in 62%, 40%, and 17% of YA patients, respectively. YAs with melanoma or cervical cancer were most likely to experience long intervals, whereas YAs with breast cancer and leukemia were most likely to experience short intervals. Conclusions: Most YAs were not seen by a cancer specialist within 2 weeks of GP consultation. Interval lengths in YAs were associated with cancer diagnosis. Patient intervals were longer among YAs than among TYAs. Our study highlights long diagnostic pathways among YAs and calls for more awareness among healthcare professionals about malignancies in this age group.</p

Therapeutic drug monitoring to personalize dosing of imatinib, sunitinib, and pazopanib:A mixed methods study on barriers and facilitators

Background:Personalized dosing based on measurement of individual drug levels and adjusting the dose accordingly can improve efficacy and decrease unnecessary toxicity of oncological treatment. For imatinib, sunitinib, and pazopanib, this therapeutic drug monitoring (TDM)-guided dosing is, however, not routinely used, despite accumulating evidence favoring individualized dosing. Therefore, we aimed to identify and quantify (potential) barriers and facilitators in TDM-guided dosing for imatinib, sunitinib, and pazopanib. Methods: We performed a mixed methods study among all stakeholders involved: patients, healthcare professionals (HCPs), pharmaceutical companies, and health insurance companies. During the first qualitative part of this study, we performed semi-structured individual interviews and one focus group interview to identify all (potential) barriers and facilitators, and during the second quantitative part of this study, we used a web-based survey to quantify these findings. The interviews addressed the six domains of the implementation of change model of Grol and Wensing: (1) the innovation itself; (2) the HCP; (3) the patient; (4) social context; (5) organizational context; and (6) finances, law, and governance. Results: In the qualitative study, we interviewed 20 patients, 18 HCPs and 10 representatives of pharmaceutical and health insurance companies and identified 72 barriers and 90 facilitators. In the quantitative study, the survey was responded by 66 HCPs and 58 patients. Important barriers were on the domain of the HCP, such as a lack of experience with TDM (36.4%), on the domain of the patient, such as lack of awareness of TDM (39.7%), and the processing time for measurement and interpretation of the TDM result (40.9%) (organizational domain). Important facilitators were education of HCPs (95.5%), education of patients (87.9%) and facilitating an overview of when and where TDM measurements are being performed (86.4%). Conclusion: We identified and quantified important barriers and facilitators for the implementation of TDM-guided dosing for imatinib, sunitinib, and pazopanib. Based on our results, the implementation strategy should mainly focus on educating both HCPs and patients and on the organizational aspect of TDM.</p

Quality of life of parents with children living at home: when one parent has cancer

Goals of work This study examined the quality of life (QoL) of cancer patients diagnosed 1-5 years previously and their spouses, with children 4-18 years living at home. Relationships between parents' QoL and the children's functioning were explored. Patients and methods 166 cancer patients and their spouses provided information on their QoL (RAND-36) and on their children's functioning (Child Behavior Checklist). Main results Male and female patients scored similarly to a norm population on five domains. Patients' QoL was clinically relevantly and/or statistically lower on social functioning, role limitations because of physical problems, and vitality than the norm. Male spouses' QoL was comparable to the norm. However, female spouses reported better physical functioning but more social problems. QoL varied according to type of cancer, treatment intensity, and recurrence. Using the QoL composite scores, a significant relationship was found between patients' psychosocial and physical functioning and spouses' psychosocial functioning. Patients' psychosocial functioning correlated moderately strongly to weakly with their reports of their younger children's and adolescents' functioning; physical functioning correlated only weakly with adolescents' functioning. The patients' functioning related weakly to moderately strongly to adolescents' self-reports of functioning. Spouses' psychosocial functioning weakly related to their and adolescents' reports of adolescents' functioning. Conclusion Cancer patients' QoL 1-5 years after diagnosis was decreased in three of eight domains; their spouses seem to be doing well. Parents' physical and psychosocial functioning related weakly to moderately strongly to their children's functioning, depending on the child's age and information source. The patients' functioning related more strongly to the children's functioning than the spouses' did

The association of having a monitoring or blunting coping style with psychological distress, health-related quality of life and satisfaction with healthcare in gastrointestinal stromal tumour (GIST) patients

Background: There are two main coping styles regarding information seeking under medical threat; monitoring (information-seeking) and blunting (information-avoiding). The aim of this study is to (1) determine factors associated with a monitoring or blunting coping style in gastro-intestinal stromal tumour (GIST) patients and (2) investigate its association with psychological distress, cancer-related concerns, health-related quality of life and satisfaction with healthcare. Methods: In a cross-sectional study, Dutch GIST patients completed the shortened version of the Threatening Medical Situations Inventory to determine their coping style, the Hospital Anxiety and Depression Scale, Cancer Worry Scale, EORTC QLQ-C30 and part of the EORTC QLQ-INFO25. Results: A total of 307 patients were classified as blunters (n = 175, 57%) or monitors (n = 132, 43%). Coping style was not associated with tumour or treatment variables, but being a female (OR 2.5; 95%CI 1.5–4.1; p= &lt;.001) and higher educated (OR 5.5; 95%CI 2.5–11.9, p= &lt;.001) were associated with higher odds of being a monitor. Monitors scored significantly lower on emotional functioning (mean = 86.8 vs mean = 90.9, p=.044), which is considered a trivial difference, more often experienced severe fear of cancer recurrence or progression (53.0% vs 37.7%, p=.007), and had more concerns about dying from GIST in the future (60.6% vs 47.4%, p=.025). Compared to blunters, monitors were less satisfied with the received healthcare and information, and would have liked to receive more information.Conclusion: GIST patients with a monitoring coping style experience a higher emotional burden. Additionally, monitors exhibit a greater need for information. Although this need for information could potentially result in fears and concerns, recognising it may also create an opening for tailored communication and information.</p

Health-related quality of life after isolated limb perfusion compared to extended resection, or amputation for locally advanced extremity sarcoma:Is a limb salvage strategy worth the effort?

Introduction: The aim of this study was to compare long-term patient reported outcomes (PROs) in patients with locally advanced extremity soft tissue sarcoma (eSTS) after isolated limb perfusion followed by resection (IR), compared to extended resection (ER), primary amputation (A) or secondary amputation after IR (IR-A). Methods: Patients were selected from the respondents of a multi-institutional cross-sectional cohort survivorship study (SURVSARC) conducted among sarcoma survivors registered in the Netherlands Cancer Registry (NCR), 2–10 years after diagnosis. Used PROs were the EORTC QLQ-C30, the Cancer worry scale (CWS), the Hospital Anxiety and Depression Scale (HADS), and the Toronto Extremity Salvage Score (TESS). Results: We identified 97 eSTS survivors: IR = 20, ER = 49, A = 20, IR-A = 8. While there were no differences in PROs between IR and ER, results showed better functioning and functionality in both groups versus the amputation groups. The amputation groups scored significantly lower on physical functioning (A = 62.7, IR-A = 65.7 versus IR = 78.0, ER = 82.7, p = 0.001) and role functioning (A = 67.5, IR-A = 52.8 versus IR = 79.2, ER = 80.6, p = 0.039), both EORTC QLQ-C30 scales. Also for the TESS, the scores were significantly lower for the amputation groups compared to the limb sparing groups (upper extremity p = 0.007 with A = 68.9, IR-A = 71.6 versus IR = 93.3, ER = 91.1; lower extremity p < 0.001 with A = 72.2, IR-A50.9 versus IR = 84.5 and ER = 85.5). There were no significant differences between the groups on cancer worry, anxiety and depression. Conclusion: HRQoL in eSTS survivors treated with IR or ER is equal; for maintenance of physical functioning and functionality IR and ER outperform an amputation

Treatment dilemmas in patients with gastrointestinal stromal tumors (GIST) who experienced imatinib-induced pneumonitis:A case series

Introduction: Imatinib has led to a phenomenal progress in the treatment of GIST. A rare and lesser-known side effect of imatinib is pneumonitis, an uncommon multicausal interstitial lung disease. Methods: Patients registered within the Dutch GIST Registry (DGR) were reviewed. For the patients identified with an imatinib-induced pneumonitis we reported the time on imatinib to develop pneumonitis, how the pneumonitis was diagnosed, graded and managed, and how the GIST treatment was managed. Cases: Of the 1934 patients registered in the DGR, 1161 patients received imatinib at some point, of which nine patients (0.8 %) were identified with an imatinib-induced pneumonitis. At time of the pneumonitis, patients received a daily imatinib dose of 200–400 mg for a mean duration of 486 days. One patient was able to continue imatinib in a lower dose, in the other eight patients imatinib was interrupted, and six of these patients started prednisolone treatment. After management of the imatinib-induced pneumonitis, four patients stopped imatinib permanently, two patients were rechallenged with imatinib, and two patients started treatment with second-line sunitinib. Conclusion: Imatinib-induced pneumonitis is a rare side effect, which may affect GIST management considerably. After the management of imatinib-induced pneumonitis, clinicians are left with difficult treatment dilemmas.</p