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.
The lengths of the patient interval and diagnostic interval are found to be prolonged in a substantial percentage of the sarcoma survivorship population in the current study. The findings highlight the need for longitudinal research among patients newly diagnosed with sarcoma to develop early diagnosis strategies for these individuals.
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 ...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.
More than one‐quarter of adolescent sarcoma survivors experience impaired social functioning 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.
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.
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.
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;
= 0.001); having ≥1 comorbidities and receiving any treatment other than surgery alone with 1.5 (95% CI 1.07-2.05;
= 0.017) and 1.4 (95% CI 1.06-1.98;
= 0.020) higher odds, respectively. Patients on active follow-up had 1.7 higher odds (95% CI 1.20-2.61;
= 0.004) and patients with higher levels of FCR scored lower on the global health status scale (72 vs. 83
≤ 0.001).
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.
Background: Sarcoma patients often experience a long time to diagnosis, known as the total interval. This interval can be divided into the patient (time from symptoms to doctor consultation) and ...diagnostic intervals (time from first consultation to diagnosis). In other cancers, a long total interval has been associated with worse outcomes, but its effect on health-related quality of life (HRQoL) has never been investigated among sarcoma patients. This study investigates the association between (1) the actual time to diagnosis and HRQoL; (2) the perceived impact of the diagnostic interval length and HRQoL; (3) the actual length and perceived impact of the length and the HRQoL of sarcoma survivors. Methods: A cross-sectional study was performed among sarcoma patients aged ≥18, diagnosed 2–10 years ago in the Netherlands. The participants completed a questionnaire on HRQoL, the time to diagnosis, the perceived impact of the diagnostic interval on HRQoL, and coping. Results: 1099 participants were included (response rate, 58%). The mean time since diagnosis was 67.4 months. More than half reported a patient (60%) or diagnostic interval (55%) ≥1 month. A third (31%) perceived a negative impact of the diagnostic interval length on HRQoL. Patient or diagnostic interval length was not associated with HRQoL. By contrast, participants perceiving a negative impact (32%) had lower HRQoL scores than those perceiving a positive (11%) or no impact (58%) (p = 0.000). This association remained significant in a multivariable model, in which maladaptive coping strategies and tumour characteristics were also found to be associated with HRQoL. Participants perceiving a negative impact of the length of the diagnostic interval related this to high psychological distress levels, more physical disabilities, and worse prognosis. Conclusion: The perceived impact of the diagnostic interval length was associated with the HRQoL of sarcoma survivors, whereas the actual length was not associated with HRQoL. Maladaptive coping strategies were independently associated with HRQoL. This offers opportunities for early intervention to improve HRQoL.
Despite curative intents of treatment in localized malignant peripheral nerve sheath tumours (MPNSTs), prognosis remains poor. This study investigated survival and prognostic factors for overall ...survival in non-retroperitoneal and retroperitoneal MPNSTs in the Netherlands.
Data were obtained from the Netherlands Cancer Registry and the Dutch Pathology Database. All primary MPNSTs were collected. Paediatric cases (age ≤18 years) and synchronous metastases were excluded from analyses. Separate Cox proportional hazard models were made for retroperitoneal and non-retroperitoneal MPNSTs.
A total of 629 localized adult MPNSTs (35 retroperitoneal cases, 5.5%) were included for analysis. In surgically resected patients (88.1%), radiotherapy and chemotherapy were administered in 44.2% and 6.7%, respectively. In retroperitoneal cases, significantly less radiotherapy and more chemotherapy were applied. In non-retroperitoneal MPNSTs, older age (60+), presence of NF1, size >5 cm, and deep-seated tumours were independently associated with worse survival. In retroperitoneal MPNSTs, male sex and age of 60+ years were independently associated with worse survival. Survival of R1 and that of R0 resections were similar for any location, whereas R2 resections were associated with worse outcomes. Radiotherapy and chemotherapy administrations were not associated with survival.
In localized MPNSTs, risk stratification for survival can be done using several patient- and tumour-specific characteristics. Resectability is the most important predictor for survival in MPNSTs. No difference is present between R1 and R0 resections in both retroperitoneal and non-retroperitoneal MPNSTs. The added value of radiotherapy and chemotherapy is unclear.
•Resectability is the most prognostic factor for survival in localized MPNSTs.•In MPNSTs, NF1, older age, tumour size, and depth are associated with poorer survival.•Retroperitoneal MPNST is a small subgroup with a worse prognosis.•R0 and R1 resections have similar survival in MPNSTs of any location.•The added value of radiotherapy and chemotherapy is unclear for survival in MPNSTs.
Plaque constitution on computed tomography coronary angiography (CTA) is associated with prognosis. At present only visual assessment of plaque constitution is possible. An accurate automatic, ...quantitative approach for CTA plaque constitution assessment would improve reproducibility and allows higher accuracy. The present study assessed the feasibility of a fully automatic and quantitative analysis of atherosclerosis on CTA. Clinically derived CTA and intravascular ultrasound virtual histology (IVUS VH) datasets were used to investigate the correlation between quantitatively automatically derived CTA parameters and IVUS VH. A total of 57 patients underwent CTA prior to IVUS VH. First, quantitative CTA quantitative computed tomography (QCT) was performed. Per lesion stenosis parameters and plaque volumes were assessed. Using predefined HU thresholds, CTA plaque volume was differentiated in 4 different plaque types necrotic core (NC), dense calcium (DC), fibrotic (FI) and fibro-fatty tissue (FF). At the identical level of the coronary, the same parameters were derived from IVUS VH. Bland–Altman analyses were performed to assess the agreement between QCT and IVUS VH. Assessment of plaque volume using QCT in 108 lesions showed excellent correlation with IVUS VH (r = 0.928,
p
< 0.001) (Fig.
1
). The correlation of both FF and FI volume on IVUS VH and QCT was good (r = 0.714,
p
< 0.001 and r = 0.695,
p
< 0.001 respectively) with corresponding bias and 95 % limits of agreement of 24 mm
3
(−42; 90) and 7.7 mm
3
(−54; 70). Furthermore, NC and DC were well-correlated in both modalities (r = 0.523,
p
< 0.001) and (r = 0.736,
p
< 0.001). Automatic, quantitative CTA tissue characterization is feasible using a dedicated software tool.
Fig. 1
Schematic illustration of the characterization of coronary plaque on CTA: cross-correlation with IVUS VH. First, the 3-dimensional centerline was generated from the CTA data set using an automatic tree extraction algorithm (
Panel I
). Using a unique registration a complete pullback series of IVUS images was mapped on the CTA volume using true anatomical markers (
Panel II
). Fully automatic lumen and vessel wall contour detection was performed for both imaging modalities (
Panel III
). Finally, fusion-based quantification of atherosclerotic lesions was based on the lumen and vessel wall contours as well as the corresponding reference lines (estimate of normal tapering of the coronary artery), as shown in
panel IV
. At the level of the minimal lumen area (MLA) (
yellow lines
), stenosis parameters, could be calculated for both imaging techniques. Additionally, plaque volumes and plaque types were derived for the whole coronary artery lesion, ranging from the proximal to distal lesion marker (
blue markers
). Fibrotic tissue was labeled in
dark green
, Fibro-fatty tissue in
light green
, dense calcium in
white
and necrotic core was labeled in
red
•Coronary artery calcium assessment on routine chest computed tomography in patients with stable chest pain.•Is a cost-free and radiation-free source of information on patients’ cardiac risk.•Has ...equal discriminative power for risk estimation of coronary artery calcium to that of cardiac directed computed tomography.•Can be used as an additional tool for clinical evaluation of patients with cardiac disease.
Given current pretest probability (PTP) estimations tend to overestimate patients’ risk for obstructive coronary artery disease, evaluation of patients’ coronary artery calcium (CAC) is more precise. The value of CAC assessment with the Agatston score on cardiac computed tomography (CT) for risk estimation has been well indicated in patients with stable chest pain. CAC can be equally well assessed on routine non–gated chest CT, which is often available. This study aims to determine the clinical applicability of CAC assessment on non–gated CT in patients with stable chest pain compared with the classic Agatston score on gated CT. Consecutive patients referred for evaluation of the Agatston score, who had a previously performed non–gated chest CT for evaluation of noncardiac diseases, were included. CAC on non–gated CT was ordinally scored. Subsequently, patients were stratified according to CAC severity and PTP. The agreement and correlation between the classic Agatston score and CAC on non–gated CT were evaluated. The discriminative power for risk reclassification of both CAC assessment methods was assessed. Invasive coronary angiography was used as the gold standard, when available. A total of 140 patients aged between 30 and 88 years were included. The agreement between ordinally scored CAC and the Agatston score was excellent (κ = 0.82) and the correlation strong (r = 0.94). Most patients (80%) with an intermediate PTP had no or mild CAC on non–gated CT. They were reclassified at low risk with 100% accuracy compared with invasive coronary angiography. Similarly, 86% of patients had an Agatston score <300. These patients were reclassified with 98% accuracy. In patients with high PTP, the accuracy remained substantial and comparable, 94% and 89%, respectively. In conclusion, we believe this is the first study to assess the clinical applicability of CAC on non–gated CT in patients with stable chest pain, compared with the classic Agatston score. The agreement between methods was excellent and the correlation strong. Furthermore, CAC assessment on non–gated CT could reclassify patients’ risk for obstructive coronary artery disease as accurately as could the classic Agatston score.
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).
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).
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.
HRQoL in eSTS survivors treated with IR or ER is equal; for maintenance of physical functioning and functionality IR and ER outperform an amputation.
The aim of the study is to assess the effect of perioperative chemotherapy (CTX) in patients with grade II-III extremity soft tissue sarcoma (eSTS) on overall survival (OS) and evaluate whether the ...PERSARC prediction tool could identify patients with eSTS more likely to benefit from CTX.
Patients (18–70 years) with primary high-grade eSTS surgically treated with curative intent were included in the retrospective cohort study. The effect of any perioperative CTX and anthracycline + ifosfamide (AI)-based CTX on OS was investigated in three PERSARC-risk groups (high/intermediate/low). The PERSARC-risk groups were defined by the 33% and 66% quantile of the predicted 5-year OS of the study population equal to a 5-year OS of 65.8% and 79.8%, respectively. The effect of CTX on OS was investigated with weighted Kaplan–Meier curves and multivariable Cox models with an interaction between risk group and CTX.
This study included 5683 patients. The weighted Kaplan-Meier curves did not demonstrate a beneficial effect of any CTX and AI-based CTX on OS in the overall population. However, in the high PERSARC-risk group the 5-year OS of AI-based CTX was significantly better than no CTX (69.8% vs 59.0%, respectively, p = 0.004) (HR 0.66, 95%CI 0.53–0.83).
This study demonstrated a beneficial effect of AI-based CTX on OS in a selected group of high-risk patients with an absolute survival benefit of 11% as stratified by the PERSARC prediction tool. However, no beneficial effect of CTX on OS was found in the overall population of patients with primary high-grade eSTS younger than 70 years.
•A selected group of patients with primary high-grade extremity soft tissue sarcoma benefits from antrhacycline+ifosfamide (AI)-based perioperative chemotherapy (CTX).•The absolute 5-year survival benefit of AI-based CTX in the high-risk group is 11%.•The PERSARC tool could identify this high-risk group with a predicted 5-year OS ≤ 66%.•There is no survival benefit of any CTX in the entire population of high-grade extremity soft tissue sarcoma.
Guidelines recommend standard pre-operative cardiac screening in all liver transplantation (LT) recipients, despite the relatively low prevalence of obstructive coronary artery disease. Most LT ...recipients often have non-gated computed tomography (CT) performed of the chest and abdomen. This study evaluated the ability of coronary artery calcification (CAC) assessment on consecutively available scans, to identify a selection of low-risk patients, in whom further cardiac imaging can be safely withheld.
LT recipients with prior non-gated CT chest-abdomen were included. CAC was visually scored on a semi-quantitative ordinal scale. Stress myocardial perfusion, coronary CT angiography (CCTA) and invasive coronary angiography (ICA) were used as golden standard. The sensitivity and specificity of CAC to exclude and predict obstructive CAD were assessed. In addition, peri- and postoperative mortality and cardiac events were analyzed.
149 LT recipients (ranged 31–71 years) were included. In 75% of patients, no CAC and mild CAC could rule out obstructive CAD on CCTA and ICA with 100% certainty. The threshold of mild CAC had a sensitivity of 100% for both CCTA and ICA and a specificity of 91% and 68%, respectively. None of the patients with no or mild calcifications experienced peri- and post-operative cardiac events or died of cardiac causes.
Visual evaluation of CAC on prior non-gated CT can accurately and safely exclude obstructive CAD in LT recipients. Incorporation of these already available data can optimize cardiac screening, by safely withholding or correctly allocating dedicated cardiac imaging in LT recipients. Thereby, reducing patients' test burden and save health care expenses.
•Coronary artery calcium assessment on non-gated CT can accurately rule out obstructive coronary artery disease in liver transplantation (LT) recipients.•In LT recipients without coronary calcium or mild calcifications additional imaging for cardiac pre-operative screening can safely be withheld.•In LT recipients with severe calcifications performance of stress myocardial perfusion or direct invasive coronary angiography is most optimal.•The incorporation of coronary artery assessment on non-gated CT can optimize cardiac pre-operative screening and save healthcare expenses.
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