Showing papers by "Otto Visser published in 2021"

Ultra-rare sarcomas: A consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities

Abstract: Background Among sarcomas, which are rare cancers, many types are exceedingly rare; however, a definition of ultra-rare cancers has not been established. The problem of ultra-rare sarcomas is particularly relevant because they represent unique diseases, and their rarity poses major challenges for diagnosis, understanding disease biology, generating clinical evidence to support new drug development, and achieving formal authorization for novel therapies. Methods The Connective Tissue Oncology Society promoted a consensus effort in November 2019 to establish how to define ultra-rare sarcomas through expert consensus and epidemiologic data and to work out a comprehensive list of these diseases. The list of ultra-rare sarcomas was based on the 2020 World Health Organization classification, The incidence rates were estimated using the Information Network on Rare Cancers (RARECARENet) database and NETSARC (the French Sarcoma Network's clinical-pathologic registry). Incidence rates were further validated in collaboration with the Asian cancer registries of Japan, Korea, and Taiwan. Results It was agreed that the best criterion for a definition of ultra-rare sarcomas would be incidence. Ultra-rare sarcomas were defined as those with an incidence of approximately ≤1 per 1,000,000, to include those entities whose rarity renders them extremely difficult to conduct well powered, prospective clinical studies. On the basis of this threshold, a list of ultra-rare sarcomas was defined, which comprised 56 soft tissue sarcoma types and 21 bone sarcoma types. Conclusions Altogether, the incidence of ultra-rare sarcomas accounts for roughly 20% of all soft tissue and bone sarcomas. This confirms that the challenges inherent in ultra-rare sarcomas affect large numbers of patients.

Primary therapy and relative survival in classical Hodgkin lymphoma: a nationwide population-based study in the Netherlands, 1989-2017.

Abstract: Population-based studies of classical Hodgkin lymphoma (cHL) in contemporary clinical practice are scarce. The aim of this nationwide population-based study is to assess trends in primary therapy and relative survival (RS) during 1989–2017. We included 9,985 patients with cHL. Radiotherapy alone was virtually not applied as from 2000 among patients aged 18–69 years with stage I/II disease, following the broader application of chemotherapy combined with radiotherapy. Chemotherapy only was the preferred treatment for patients with stage III/IV disease. Throughout the entire study period, around 20% of patients aged ≥70 years across all disease stages received no anti-neoplastic therapy. The most considerable improvements in 5-year RS were confined to patients aged 18–59 years. Five-year RS for patients with stage I/II disease diagnosed during 2010–2017 was 99%, 98%, 100%, 93%, 84%, and 61% for patients aged 18–29, 30–39, 40–49, 50–59, 60–69, and ≥70 years, respectively. The corresponding estimates for stage III/IV disease were 96%, 92%, 90%, 80%, 58%, and 46%. Collectively, the improvements in survival likely relate to advances in cHL management. These achievements, however, do not seem to translate into significant benefits for patients ≥60 years. Therefore, novel therapies are urgently needed to reduce excess mortality in elderly cHL patients.

Stage-specific trends in primary therapy and survival in follicular lymphoma : a nationwide population-based analysis in the Netherlands, 1989–2016

Abstract: We assessed stage-specific trends in primary therapy and relative survival among adult follicular lymphoma (FL) patients diagnosed in the Netherlands between 1989–2016 (N = 12,372; median age, 62 years; and 21% stage I disease). Patients were stratified by disease stage and subsequently categorized into four calendar periods (1989–1995, 1996–2002, 2003–2008, and 2009–2016) and three age groups (18–60, 61–70, and >70 years). The use of radiotherapy in stage I FL remained relatively stable over time and across the three age groups (i.e., 66%, 54%, and 49% in 2009–2016, respectively). In stage II-IV FL, the start of chemotherapy within 12 months post-diagnosis decreased over time, indicating a broader application of a watch-and-wait approach. Relative survival improved considerably over time, especially since 2003 when rituximab was introduced in the Netherlands, and for stage III-IV FL patients and older age groups. Five-year relative survival for patients with stage I-II versus stage III-IV FL in the period 2009–2016 was 96% versus 90%, 93% versus 83%, and 92% versus 68% across the three age groups, respectively. Collectively, the improvement in survival since 2003 is accounted for by advances in FL management, particularly the implementation of rituximab. There remains, however, room for improvement among elderly stage III-IV FL patients.

Primary therapy and relative survival in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia: a population-based study in the Netherlands, 1989-2018.

Abstract: It is unclear how treatment advances impacted the population-level survival of patients with lymphoplasmacytic lymphoma/Waldenstrom macroglobulinaemia (LPL/WM). Therefore, we assessed trends in first-line therapy and relative survival (RS) among patients with LPL/WM diagnosed in the Netherlands between 1989 and 2018 (N = 6232; median age, 70 years; 61% males) using data from the nationwide Netherlands Cancer Registry. Patients were grouped into three age groups ( 75 years) and four calendar periods. Overall, treatment with anti-neoplastic agents within 1 year post-diagnosis gradually decreased over time, following a broader application of an initial watch-and-wait approach. Approximately 40% of patients received anti-neoplastic therapy during 2011-2018. Furthermore, use of chemotherapy alone decreased over time, following an increased application of chemoimmunotherapy. Detailed data among 1596 patients diagnosed during 2014-2018 revealed that dexamethasone-rituximab-cyclophosphamide was the most frequently applied regimen; its use increased from 14% to 39% between 2014 and 2018. The 5-year RS increased significantly over time, particularly since the introduction of rituximab in the early-mid 2000s. The 5-year RS during 1989-1995 was 75%, 65%, and 46% across the age groups compared to 93%, 85%, and 79% during 2011-2018. However, the survival improvement was less pronounced after 2011. Collectively, the impressive survival improvement may be accounted for by broader application of rituximab-containing therapy. The lack of survival improvement in the post-rituximab era warrants studies across multiple lines of therapy to further improve survival in LPL/WM.

A population-based study on different regimens of R-CHOP in patients with newly diagnosed DLBCL in The Netherlands.

Abstract: Randomized controlled trials have studied different dose-intensity and dose-interval regimens of R-CHOP for patients with diffuse large B-cell lymphoma (DLBCL). This study was undertaken to confirm these results in a population-based setting, with special emphasis on the value of 6xR-CHOP21 among patients aged 18–64 years. Two thousand three hundred and thirty-eight stage II–IV DLBCL patients, ≥18 years, we confirmed the similar efficacy of six versus eight cycles of R-CHOP and of R-CHOP21 versus R-CHOP14 regimens across all age groups on overall survival (median follow-up 36.4 (1.3–167.6) months). Nevertheless, overall survival decreased with older age. Interestingly, in patients 18–64 years, the adjusted risk of mortality among recipients of 6xR-CHOP21 compared to other R-CHOP regimens seems to be similar (HR 0.62; 95%CI: 0.38–1.02; p= .059). Although this finding might suggest that 6xR-CHOP21 could be considered as first-line regimen for all stage II–IV DLBCL patients, it should be confirmed in forthcoming population-based studies with larger patient numbers and longitudinal follow-up.

Reply to: "Discussing sexuality in cancer care: towards personalized information for cancer patients and survivors".

Abstract: To the Editor, We have read with interest the manuscript “Discussing sexuality in cancer care: towards personalized information for cancer patients and survivors” by Albers et al. [1]. In a cross-sectional survey among members of cancer patient organizations, the authors investigated the patients’ need for information about sexuality and how to improve communication and aimed to identify “who really wants to know.” The authors concluded that 65% of members had a need for information regarding sexuality. Younger patients, patients with a self-reported negative effect of cancer on sexuality, and patients who were diagnosed less than 2 years ago reported a greater need for information. Most participants were treated for solid cancers. Although we applaud Albers’ work, we believe that we should address the needs of the patients’ partners as well; therefore, we performed a multicenter longitudinal survey to measure the need for information not only among patients with hematological malignancies (HM) during the first 18 months of treatment but also among their partners. To identify information needs on sexuality, six questions were developed for this study. In addition to the results of Albers et al., we here report not only cross-sectional data but also longitudinal data of information needs on sexuality of patients and their partners as the need for information might well change over time. This prospective survey was conducted in the Netherlands between 2013 and 2015. Adult patients (n = 344) diagnosed with HM and their partners were invited to participate directly after diagnosis (T0), 3 months (T1), 6 months (T2), 1 year (T3), and 18 months after diagnosis (T4). With regard to sexuality, the Sexual and Fertility Information Needs Questionnaire (SFINFOQ) is comprised of 6 items on information needs regarding (1) changes in sexuality (3 items), (2) support for sexual difficulties (2 items), and (3) precautions that need to be taken (1 item). The response format of the items was a 5-point Likert scale (range: not important to extremely important). The responses “very important” and “extremely important”were identified as an informational need at that time. Furthermore, the survey included items on possible factors that might influence information needs. Except for age (categorized in clinically relevant blocks: adolescents and young adults < 36 years, adults 36–69 years, older adults ≥ 70 years), education (low, middle, and high), and diagnosis (per disease), all other sociodemographic and health status variables were dichotomized. A multivariable model was built with a forward selection procedure using generalized estimating equations, to account for the longitudinal data collection (p value for entry < 0.05). All data were analyzed using IBM SPSS 26 statistics. The study was approved by the Ethics Committee of the VU University Medical Center, Amsterdam, the Netherlands. Written informed consent was obtained from all patients. In total, the survey was completed by 266 patients and 134 partners at one or more time points. The mean age of the patients at time of diagnosis was 58 years (SD 14.9). Over 18 months from diagnosis, range 42 (at T0) to 48% (at T3) of patients expressed a need for information regarding changes in sexuality, 40 (T1) to 44% (T3) expressed a need for support for sexual difficulties, and 41 (T3) to 46% (T0) expressed a need for precautions that need to be taken. Multivariable analyses (Fig. 1) revealed that information needs of patients regarding changes in sexuality and precautions to be taken were associated with younger age, male sex, and a monitoring cognitive coping style. A need for support was associated with younger age and a monitoring coping style. Regarding their partners, 34% (T4) to 52% (T3) expressed a need for information regarding changes in sexuality, 39% * Corien M. Eeltink C.Eeltink@vumc.nl

Conditional relative survival in Waldenström's macroglobulinaemia: a population-based study in The Netherlands.

Abstract: Contemporary diagnosed WM patients, compared to the general population, continue to experience excess mortality regardless of having survived up to 15 years post-diagnosis. This gradual increase in excess mortality might result from the incurable nature of this disease characterized by multiple relapses throughout the disease course with limited efficacious treatment options in the released/refractory setting.