Quality of life of patients with rare cancer: a comparison with patients with colorectal cancer and the association with disease trajectory-related factors

Purpose: Differences in quality of life (QoL) between patients with rare and common cancer might be explained by the specific challenges patients with rare cancer face during their disease trajectory, but research is scarce. This study aimed to (1) assess the difference in QoL between patients with rare and common cancer (i.e. colorectal cancer (CRC)) and (2) examine the association between disease trajectory-related factors and QoL in patients with rare cancer. Methods: Cross-sectional data were collected among adults with rare cancer by a nationwide online survey in the Netherlands. For comparison with patients with CRC, data from the Prospective Dutch Colorectal Cancer (PLCRC) cohort were used. Associations were assessed by linear regression analyses. Results: Data from 1525 patients with rare cancer and 1047 patients with CRC were analysed. Having a rare cancer was significantly associated with a lower QoL compared to having CRC (p < 0.001). Disease trajectory-related factors significantly associated with QoL in patients with rare cancer were time until diagnosis, misdiagnoses, information on best treatment options, information on late and/or long-term effects, and both satisfaction with physician and specialized nurse care (all: p < 0.05). Conclusion: Patients with rare cancers have a lower self-reported QoL than patients with CRC, and several disease trajectory-related factors are associated with QoL in patients with rare cancer. Implications for Cancer Survivors: To improve QoL of patients with rare cancer, appropriate guidance and support by healthcare professionals throughout the disease trajectory are needed, as well as early diagnosis and proper referral to centres of expertise

Unmet supportive care needs of patients with rare cancer: A systematic review

Objective: Patients with rare cancers may experience different unmet needs than those with common cancer. The objective of this systematic review was to (1) investigate unmet supportive care needs of rare cancer patients throughout the disease trajectory and (2) identify predictive factors for these unmet needs. Methods: PubMed, PsycINFO and CINAHL were searched for publications (January 2011 to March 2021) focusing on unmet needs of patients with rare cancer. Two reviewers independently selected studies, extracted data and performed quality assessment. Findings were synthesised. Results: The search yielded 4,598 articles, of which 59 articles met eligibility criteria and 57 were of medium or high quality. Rare cancer patients most frequently reported unmet needs in the healthcare system and information domain (up to 95%), followed by the psychological domain (up to 93%) and the physical and daily living domain (up to 80%). Unmet needs were mainly reported in the posttreatment phase. The most frequently identified predictors were higher anxiety, younger age and higher neuroticism. Conclusion: Patients with rare cancer have unmet needs throughout their disease trajectory. Supportive care needs of rare cancer patients should be addressed individually, depending on the rare cancer subdomain and phase of disease and from diagnosis onwards

Challenges and controversies patients and (health care) professionals experience in managing vaginal, vulvar, penile or anal cancer: The SILENCE study

Objective: Patients with vaginal, vulvar, penile or anal cancer experience deteriorated psychosocial functioning and decreased Quality of Life (QoL). The aims of this study were to explore (1) the challenges and controversies patients experience in managing vaginal, vulvar, penile or anal cancer; their unmet needs; and how this affects their psychosocial functioning and (2) the gaps health care professionals (HCPs) experience in providing psychosocial support and potential improvements in care. Methods: Semi-structured interviews with patients with vaginal, vulvar, penile or anal cancer and with HCPs were conducted. All interviews were transcribed verbatim and thematically analysed. Results: Fourteen patients (86% female; mean age 55.5) and 12 HCPs (75% female; mean age 46.4) participated. Four themes were identified: (1) recognisable symptoms but unfamiliar diagnosis, (2) ‘double hit’ has severe impact on psychosocial functioning, (3) personal and tailored information is important but not guaranteed and (4) all-encompassing care to improve psychosocial functioning and QoL. Conclusion: Patients with vaginal, vulvar, penile or anal cancer encounter a lack of awareness and knowledge about their rare cancer type, difficulties regarding communication and long-term changes in body image and sexuality. Awareness of symptoms should be raised and psychosocial care should be offered on a structural basis

The gap between rare and common cancers still exists: Results from a population-based study in the Netherlands

Introduction: Epidemiological discrepancies exist between rare and common cancers. The aim of this population-based study was to compare rare versus common adult solid cancers in the Netherlands, by providing incidence, prevalence and survival rates, evaluating trends in survival and comparing individual entities within domains and families. Methods: All adult patients with malignant solid cancers in the Netherlands between 1995 and 2019 were identified from the Netherlands Cancer Registry. Data on patient, tumour and treatment characteristics were collected, and relative survival and survival trends were analysed. Results: A total of 170,628 patients with rare adult solid cancers and 806,023 patients with common adult solid cancers were included. Rare cancers accounted for 18% of all cancer diagnoses (mean incidence), and 15% of the total ten-year cancer prevalence during 2010–2019. Overall 5-year survival was worse for rare cancers than for common cancers (52.0% versus 68.7%). Between 1995–1999 and 2015–2019, 5-year survival rates for rare cancers increased to a lesser extent (from 46.2% to 52.6%, i.e. 6.4%) than for common cancers (56.9%–70.1%, i.e. 13.2%), and for most rare cancer domains compared to common cancer domains. The majority of rare cancer entities did not show an improvement in 5-year survival. Differences for individual entities between domains and families were found. Conclusion: Differences in survival between rare and common cancers indicate major challenges for rare cancer care and emphasise that improvement is highly needed. Observed inequalities need to be overcome by investing in early diagnosis, novel therapies, scientific research and in establishing centres of expertise

Challenges and controversies patients and (health care) professionals experience in managing vaginal, vulvar, penile or anal cancer: The SILENCE study

Objective: Patients with vaginal, vulvar, penile or anal cancer experience deteriorated psychosocial functioning and decreased Quality of Life (QoL). The aims of this study were to explore (1) the challenges and controversies patients experience in managing vaginal, vulvar, penile or anal cancer; their unmet needs; and how this affects their psychosocial functioning and (2) the gaps health care professionals (HCPs) experience in providing psychosocial support and potential improvements in care. Methods: Semi-structured interviews with patients with vaginal, vulvar, penile or anal cancer and with HCPs were conducted. All interviews were transcribed verbatim and thematically analysed. Results: Fourteen patients (86% female; mean age 55.5) and 12 HCPs (75% female; mean age 46.4) participated. Four themes were identified: (1) recognisable symptoms but unfamiliar diagnosis, (2) ‘double hit’ has severe impact on psychosocial functioning, (3) personal and tailored information is important but not guaranteed and (4) all-encompassing care to improve psychosocial functioning and QoL. Conclusion: Patients with vaginal, vulvar, penile or anal cancer encounter a lack of awareness and knowledge about their rare cancer type, difficulties regarding communication and long-term changes in body image and sexuality. Awareness of symptoms should be raised and psychosocial care should be offered on a structural basis

Differences in health care experiences between rare cancer and common cancer patients: results from a national cross-sectional survey

Background: Patients with rare cancers face challenges in the diagnostic and treatment phase, and in access to clinical expertise. Since studies on health care experiences of these patients in comparison to patients with more common cancers are scarce, we aimed to explore these differences. Methods: Data were cross-sectionally collected among (former) adult cancer patients through a national online survey in the Netherlands (October 2019). Descriptive statistics were reported and subgroups (rare vs. common patients) were compared. Results: In total, 7343 patients (i.e., 1856 rare and 5487 common cancer patients) participated. Rare cancer patients were more often diagnosed and treated in different hospitals compared to common cancer patients (67% vs. 59%, p < 0.001). Rare cancer patients received treatment more often in a single hospital (60% vs. 57%, p = 0.014), but reported more negative experiences when treated in multiple hospitals than common cancer patients (14% vs. 9%, p < 0.001). They also more often received advise from their physician about the hospital to go to for a second opinion (50% vs. 36%, p < 0.001), were more likely to choose a hospital specialized in their cancer type (33% vs. 22%, p < 0.001), and were more willing to travel as long as necessary to receive specialized care than common cancer patients (55% vs. 47%, p < 0.001). Conclusions: Rare and common cancer patients differ in their health care experiences. Health care for rare cancer patients can be further improved by proper referral to centers of expertise and building a clinical network specifically for rare cancers

Differences in health care experiences between rare cancer and common cancer patients: results from a national cross-sectional survey

Background: Patients with rare cancers face challenges in the diagnostic and treatment phase, and in access to clinical expertise. Since studies on health care experiences of these patients in comparison to patients with more common cancers are scarce, we aimed to explore these differences. Methods: Data were cross-sectionally collected among (former) adult cancer patients through a national online survey in the Netherlands (October 2019). Descriptive statistics were reported and subgroups (rare vs. common patients) were compared. Results: In total, 7343 patients (i.e., 1856 rare and 5487 common cancer patients) participated. Rare cancer patients were more often diagnosed and treated in different hospitals compared to common cancer patients (67% vs. 59%, p < 0.001). Rare cancer patients received treatment more often in a single hospital (60% vs. 57%, p = 0.014), but reported more negative experiences when treated in multiple hospitals than common cancer patients (14% vs. 9%, p < 0.001). They also more often received advise from their physician about the hospital to go to for a second opinion (50% vs. 36%, p < 0.001), were more likely to choose a hospital specialized in their cancer type (33% vs. 22%, p < 0.001), and were more willing to travel as long as necessary to receive specialized care than common cancer patients (55% vs. 47%, p < 0.001). Conclusions: Rare and common cancer patients differ in their health care experiences. Health care for rare cancer patients can be further improved by proper referral to centers of expertise and building a clinical network specifically for rare cancers