The sarcoma diagnostic interval: a systematic review on length, contributing factors and patient outcomes

Soomers, Vicky L. M. N.; Husson, Olga; Young, Rowan D.; Graaf, Winette van der

doi:10.1136/esmoopen-2019-000592

Cited by 55 publications

(86 citation statements)

References 78 publications

(227 reference statements)

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“…Another possible explanation would be that the rarity of GIST at the YA age leads to a delay in (correct) diagnosis. Delay of diagnosis in sarcomas, especially at this age, remains a problem, and efforts to educate doctors and patients should be continued [35].…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry

IJzerman

Drabbe

Hollander

et al. 2020

Cancers

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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 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.

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Section: Discussionmentioning

confidence: 99%

Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry

IJzerman

Drabbe

Hollander

et al. 2020

Cancers

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“…Based on a literature review, sex, age at diagnosis, SES, histology, stage of disease, tumor grade, and localization were selected as independent variables. 4 In the case of multicollinearity, we tried both factors in different models. The factor that resulted in the best model was chosen for further analysis.…”

Section: Discussionmentioning

confidence: 99%

“…Questions regarding patient and diagnostic intervals were designed by the study group to match time intervals and events as defined in our adapted version of the standardized definitions proposed by Olesen et al and Weller et al, 5,6 and as published before. 4 The Cancer December 15, 2020 diagnostic interval can be divided further into a primary care, secondary care, and tertiary care interval. All interval lengths were categorical and patient reported (<2 weeks, 2 weeks-1 month, 1-3 months, 3-6 months, 6-12 months, and >12 months).…”

Section: Study Measuresmentioning

confidence: 99%

“…3 Patients with sarcoma may experience long intervals to diagnosis, and the time to diagnosis has been measured frequently. 4 Total intervals for patients with BS were 9 to 120.4 weeks, and were reported to be 4.3 to 614.9 weeks for patients with STS. However, these studies often described small cohorts and were heterogeneous with regard to inclusion criteria and study designs.…”

Section: Introductionmentioning

confidence: 99%

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Patient and diagnostic intervals of survivors of sarcoma: Results from the SURVSARC study

Soomers¹,

Husson

Desar³

et al. 2020

Cancer

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BACKGROUND: Patients diagnosed with sarcoma are hypothesized to experience a prolonged route to a cancer diagnosis. This route, the total interval, can be divided into a patient interval (the time from the appearance of symptoms to physician consultation) and diagnostic interval (time from the first consultation to diagnosis). In the current study, the authors investigated these intervals among survivors of sarcoma and identified factors associated with prolonged intervals. METHODS: A cross-sectional study was conducted among adult patients with sarcoma 2 to 10 years after diagnosis. Patients completed a questionnaire regarding their total interval, which was linked to clinical data from the Netherlands Cancer Registry. Descriptive statistics were used to describe intervals. Based on Dutch clinical guidelines, a diagnostic interval ≥1 month was considered to be prolonged and an interval ≥3 months was considered as very long. Multivariable regression analyses investigated associations between patient and tumor characteristics and interval length. RESULTS: A total of 1099 participants were included (response rate, 58%); approximately 60% reported a patient interval ≥1 month and 36% reported a patient interval ≥3 months. Risk factors for a very long patient interval were sarcoma of the skin or pelvis, liposarcoma, or rhabdomyosarcoma. Stage III disease was associated with a shorter patient interval. The diagnostic interval length was ≥1 month in 55% of patients and ≥3 months in 28% of patients. Risk factors for a very long diagnostic interval were female sex, age <70 years, or having a synovial sarcoma or chordoma. CONCLUSIONS: The patient and diagnostic interval lengths were prolonged in a substantial percentage of this sarcoma survivorship population. Factors found to be associated with the length of the patient interval or the diagnostic interval differed. Creating awareness among (especially young) patients to consult a physician and awareness among physicians to consider a sarcoma diagnosis will contribute to optimization of the total interval.

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“…Some studies found that a longer total interval worsened OS, while others did not find inferior clinical outcomes. 10 Researchers have argued that this lack of an association, often referred to as the ‘waiting-time paradox’, may be because the studies have not been able to adequately adjust for the aggressiveness of the cancer tumours. The most significant effect of a long interval for sarcomas seems to be the increasing size of the lesion, 12 with consequent decreased chance of uncomplicated resection with clear surgical margins, a greater risk of amputation and increased risk of developing metastases.…”

Section: Introductionmentioning

confidence: 99%

Quality of life and experiences of sarcoma trajectories (the QUEST study): protocol for an international observational cohort study on diagnostic pathways of sarcoma patients

et al. 2020

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IntroductionSarcomas are rare tumours with considerable heterogeneity. Early and accurate diagnosis is important to optimise patient outcomes in terms of local disease control, overall survival (OS) and health-related quality of life (HRQoL). Time to diagnosis is variable in bone as well as soft tissue sarcoma. Possible factors for a long time from first symptom to diagnosis (the total interval) include patient, tumour and healthcare characteristics, but until now the most relevant risk factors and its association with outcomes remain unknown. Our study aims to (1) quantify total interval, the time interval from first symptom until (histological) diagnosis; (2) identify factors associated with interval length and (3) determine the association between total interval and HRQoL, stage and tumour size at diagnosis, progression-free survival (PFS) and OS.Methods and analysisWe will conduct a longitudinal, prospective, international, multicentre cohort study among patients aged ≥18 years with newly diagnosed bone or soft tissue sarcoma at eight centres (three in UK, five in The Netherlands). Patients will be asked to complete questionnaires at five points in time; one at diagnosis and at follow-up points of 3, 6, 12 and 24 months. Questionnaire data is collected within the Patient Reported Outcomes Following Initial treatment and Long term Evaluation of Survivorship (PROFILES) registry: an international data management system for collection of patient-reported outcomes. Clinical data will be extracted from patient records. The primary endpoint is HRQoL at diagnosis, measured with the EORTC QLQ-C30. Secondary endpoints are stage and tumour size at diagnosis, PFS, OS, additional patient-reported outcomes, such as quality-adjusted life years and psychological distress.Ethics and disseminationEthical approval was given by the Health Research Authority and Research Ethics Committee for the United Kingdom (18/WA/0096) and medical ethical committee of Radboudumc for The Netherlands (2017-3881). Results will be presented in peer-reviewed journals and presented at meetings.Trial registration numberNCT03441906.

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The sarcoma diagnostic interval: a systematic review on length, contributing factors and patient outcomes

Cited by 55 publications

References 78 publications

Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry

Gastrointestinal Stromal Tumours (GIST) in Young Adult (18–40 Years) Patients: A Report from the Dutch GIST Registry

Patient and diagnostic intervals of survivors of sarcoma: Results from the SURVSARC study

Quality of life and experiences of sarcoma trajectories (the QUEST study): protocol for an international observational cohort study on diagnostic pathways of sarcoma patients

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