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Desmoid tumor (DT; other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously surgery was the standard primary treatment modality; however, in recent years a paradigm shift towards a more conservative management has been introduced and an effort to harmonise the strategy amongst clinicians has been made. We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients Euro-Net (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought

Background Non-adherence negatively affects the efficacy, safety and costs of therapies. Non-adherence is a multifactorial problem. This systematic review (SR) of SRs (overview) aims to identify factors that can influence the adherence of adult patients with chronic physical diseases. Methods We performed a systematic literature search in MEDLINE and Embase on June 13, 2018. We included SRs on the factors that can influence adherence in adult patients taking oral medications for treating physical chronic diseases. Two reviewers independently selected studies according to pre-defined inclusion criteria. Two reviewers independently assessed the risk of bias with the ROBIS tool. Data were extracted in standardized tables previously piloted by one reviewer and verified by a second reviewer. We synthesized data in tables in a structured narrative manner. Results We included 21 SRs on eight different conditions. We rated eight SRs to be at low risk of bias and 13 to be at high risk of bias. Although higher education, employment, higher financial status and marriage/partnership mostly showed a positive effect on adherence, the impact was unclear because of the high uncertainty of the underlying evidence. The evidence indicates that socioeconomic status and social support might have a positive impact on adherence and that belonging to an ethnic minority might have a negative impact on adherence. Therapy-related factors (e.g., intake regime) and disease-related factors (e.g., duration) mostly showed no impact on adherence. Analysis of gender showed inconsistent results. Age might have a concave relation to adherence, i.e., adherence is lowest in very young and very old people. Depression has a negative impact on adherence. Impacts of other mental and physical comorbidities were uncertain. Co-payments (any or higher) have a negative impact on adherence. In contrast, the impacts of medication costs and insurance status were uncertain. Conclusion This overview analyses factors that might impact adherence to oral therapies in adult patients with physical chronic diseases. Our overview suggests that there is a social gradient in adherence. However, for most factors, the evidence was not conclusive due to the risk of bias, inconsistency or imprecision. Electronic supplementary material The online version of this article (10.1186/s13643-019-1014-8) contains supplementary material, which is available to authorized users.

BackgroundThe surgeon volume-outcome relationship has been discussed for many years and its existence or nonexistence is of importance for various reasons. A lot of empirical work has been published on it. We aimed to summarize systematic reviews in order to present current evidence.MethodsMedline, Embase, Cochrane database of systematic reviews (CDSR), and health technology assessment websites were searched up to October 2015 for systematic reviews on the surgeon volume-outcome relationship. Reviews were critically appraised, and results were extracted and synthesized by type of surgical procedure/condition.ResultsThirty-two reviews reporting on 15 surgical procedures/conditions were included. Methodological quality of included systematic reviews assessed with the assessment of multiple systematic reviews (AMSTAR) was generally moderate to high albeit included literature partly neglected considering methodological issues specific to volume-outcome relationship. Most reviews tend to support the presence of a surgeon volume-outcome relationship. This is most clear-cut in colorectal cancer, bariatric surgery, and breast cancer where reviews of high quality show large effects.ConclusionsWhen taking into account its limitations, this overview can serve as an informational basis for decision makers. Our results seem to support a positive volume-outcome relationship for most procedures/conditions. However, forthcoming reviews should pay more attention to methodology specific to volume-outcome relationship. Due to the lack of information, any numerical recommendations for minimum volume thresholds are not possible. Further research is needed for this issue.Electronic supplementary materialThe online version of this article (doi:10.1186/s13643-016-0376-4) contains supplementary material, which is available to authorized users.

BackgroundNon-adherence is widespread problem. Adherence is a crucial point for the success and the safe use of therapies. The objective of this overview (review of reviews) was to identify factors that influence adherence in chronic physical conditions.MethodsA systematic literature search was performed in Medline and Embase (1990 to July 2013). Publications were screened according to predefined inclusion criteria. The study quality was assessed using AMSTAR. Both process steps were carried out independently by two reviewers. Relevant data on study characteristics and results were extracted in piloted standardized tables by one reviewer and checked by a second. Data were synthesized using a standardized quantitative approach by two reviewers.ResultsSeven systematic reviews were included. Higher education and employment seem to have a positive effect on adherence. Ethnic minorities seem to be less adherent. Co-payments and higher medication cost seems to have negative effect on adherence. In contrast financial status/income and marital status seem to have no influence on adherence. The effect of therapy related factors was mostly unclear or had no effect. Only the number of different medications in heart failure patients showed the tendency of an effect. Indicators of regime complexity showed consistently a negative effect direction. Duration of disease seems to have no effect on adherence. There is the tendency that higher or middle age is associated with higher adherence. But in more than half of the reviews the effect was unclear. There is no clear effect of physical as well as mental comorbidity. Only one review showed the tendency of an effect for mental comorbidity. Also for gender the effect is not clear because the effect direction was heterogenic between and within the systematic reviews.ConclusionThe presented overview shows factors than can potentially have influence on adherence. Only for a few factors the influence on adherence was consistent. Most factors showed heterogeneous results regarding statistical significance and/or effect direction. However, belonging to an ethnic minority, unemployment and cost for the patient for their medications showed consistently a negative effect on adherence which indicates that there is a social gradient.Electronic supplementary materialThe online version of this article (doi:10.1186/2049-3258-72-37) contains supplementary material, which is available to authorized users.