Extra-Pulmonary Neuroendocrine Carcinomas: A Population-Based Study in the Netherlands

2018 ◽  
Vol 107 (1) ◽  
pp. 50-59 ◽  
Author(s):  
Jan Maarten van der Zwan ◽  
Sabine Siesling ◽  
Loes van Velthuysen ◽  
Thera Links ◽  
Annemiek Walenkamp ◽  
...  

Background: Extra-pulmonary neuroendocrine carcinomas (EP-NEC) are rare tumours that require expertise for correct and timely diagnosis, which is essential for clinical decision making. The number of patients affected, treatment given, and the proportion surviving the disease is based on limited evidence. The aim of this study is to retrospectively analyse the incidence, treatment, and relative survival (RS) of EP-NEC patients in the Netherlands. Methods: Patients diagnosed between 2008–2012 with EP-NEC or NEC with unknown primary site (UP-NEC) were selected from the Netherlands Cancer Registry based on combinations of tumour localisation and morphology code. Incidence was studied using the European standardised (ESR) and world standardised rates, and RS was calculated using the Ederer II method. Results: In total, 1,544 cases were analysed, 1,045 EP-NEC and 499 UP-NEC. For EP-NEC, the incidence was 1.0 per 100,000 person-years (ESR), the mean age was 68 years, and the male to female ratio was 1: 0.6. Most frequent EP-NEC localisations were the bladder and the gastrointestinal tract, and the treatment most frequently given was surgery in combination with chemotherapy. The overall 5-year RS was 38% for patients with local/regional disease (n = 447), and 7% for patients with extensive disease (n = 582). For UP-NEC patients (n = 499), the 5-year RS was 6%. Conclusions: This study is the first nationwide study presenting an increase in the incidence of EP-NEC patients from 196 to 260 cases annually in the Netherlands. The best 5-year RS was found for EP-NEC patients with local disease located in the bladder, where the worst 5-year RS was found for patients with disease located in the oesophagus.

2013 ◽  
Vol 20 (2) ◽  
pp. 263-271 ◽  
Author(s):  
Olga Husson ◽  
Harm R Haak ◽  
Liza N van Steenbergen ◽  
Willy-Anne Nieuwlaat ◽  
Boukje A C van Dijk ◽  
...  

The incidence of thyroid cancer (TC) is increasing worldwide, partly due to increased detection. We therefore assessed combined trends in incidence, survival and mortality of the various types of TC in The Netherlands between 1989 and 2009. We included all patients ≥15 years with TC, diagnosed in the period 1989–2009 and recorded in The Netherlands Cancer Registry (n=8021). Information on age, gender, date of diagnosis, histological type of tumour and tumour–node–metastasis classification was recorded. Mortality data (up to 1st January 2010) were derived from Statistics Netherlands. Annual percentages of change in incidence, mortality and relative survival were calculated. Since 1989 the incidence of TC increased significantly in The Netherlands (estimated annual percentage change (EAPC)=+1.7%). The incidence rates increased for all age groups (except for females >60 years), papillary tumours (EAPC=+3.5%), T1 and T3 TC (EAPC=+7.9 and +5.8% respectively). Incidence rates decreased for T4 TC (−2.3%) and remained stable for follicular, medullary anaplastic and T2 TC. Five-year relative survival rates remained stable for papillary (88%) and follicular (77%) TC, all age groups and T1–T3 TC (96, 94 and 80% respectively) and somewhat lower for T4 (53%), medullary (65%) and anaplastic TC (5%) in the 2004–2009 period compared with earlier periods. Mortality due to TC decreased (EAPC=−1.9%). TC detection and incidence has been rising in The Netherlands, while mortality rates are decreasing and survival rates remained stable or slightly decreasing.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A76-A76
Author(s):  
Rebecca Steenaard ◽  
Marieke Rutjens ◽  
Harm Reinout Haak

Abstract Background: Adrenocortical carcinoma (ACC) is a rare disease with often poor prognosis. Previous research has shown that surgery in a Dutch Adrenal Network (DAN) center increases the chance of survival. We aim to explore the determinants and survival of patients with ACC recently treated in the Netherlands both within and outside DAN centers. Methods: We analyzed retrospectively collected data from 172 adult patients with newly diagnosed ACC and 97 patients with recurrence or new metastases, registered between 2014 and 2019 in the Netherlands Cancer Registry. Differences in survival were analyzed with cox-regression analysis. Results: More than half of the new cases presented with advance disease (25.7% stage III, 34.6% stage IV) and the median survival was 29 months. The majority of treatments occurred within a DAN center (87.2% of surgery, compared to 56.4% between 1999 and 2009; and 94.5% of medical treatment). There were no differences in patient characteristics between the centers apart from a relatively high number of patients with stage IV disease outside DAN centers (47.2% vs. 28.7%). Adrenal resection and mitotane therapy both resulted in a significant survival benefit (resection HR 0.29, CI95%[0.17–0.49]; mitotane HR 0.61, CI95%[0.37–0.99], corrected for stage). Still, a remarkable proportion of patients with advanced disease received no mitotane treatment (39.8%). Due to the small number of patients treated outside DAN centers, survival benefits could not be tested. Conclusions: Centralization of ACC care in the Netherlands has improved since the previous report, but a further improvement in centralization of surgery can be made. Adrenal resection and mitotane treatment remain the main form of treatment, with a clear survival benefit. Further research is necessary to determine why mitotane treatment is withheld in a large proportion of patients with advance disease.


Cancers ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3421
Author(s):  
Daniël J. van der Meer ◽  
Henrike E. Karim-Kos ◽  
Marianne van der Mark ◽  
Katja K. H. Aben ◽  
Rhodé M. Bijlsma ◽  
...  

Adolescent and young adult (AYA) cancer patients, aged 15–39 years at primary cancer diagnosis, form a distinct, understudied, and underserved group in cancer care. This study aimed to assess long-term trends in incidence, survival, and mortality of AYA cancer patients within the Netherlands. Data on all malignant AYA tumours diagnosed between 1990–2016 (n = 95,228) were obtained from the Netherlands Cancer Registry. European age-standardised incidence and mortality rates with average annual percentage change (AAPC) statistics and five-year relative survival rates were calculated. The overall cancer incidence increased from 54.6 to 70.3 per 100,000 person-years (AAPC: +1.37%) between 1990–2016, and increased for both sexes individually and for most cancer types. Five-year relative survival overall improved from 73.7% in 1990–1999 to 86.4% in 2010–2016 and improved for both sexes and most cancer types. Survival remained poor (<60%) for rhabdomyosarcoma, lung, stomach, liver, bladder, and pancreatic carcinomas, among others. Mortality rates among male AYAs overall declined from 10.8 to 6.6 (AAPC: −1.64%) and from 14.4 to 10.1 per 100,000 person-years (AAPC: −1.81%) for female AYAs since 1990. Mortality rates remained unchanged for male AYAs aged 20–24 and 25–29 years. In conclusion, over the past three decades, there has been a considerable increase in cancer incidence among AYAs in the Netherlands. Meanwhile, the survival improved and the mortality overall declined. Survival at five-years now well exceeds above 80%, but did not do so for all cancer types.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2852-2852
Author(s):  
Marek Trneny ◽  
Petra Obrtlikova ◽  
Jiri Schwarz ◽  
Tomas Pavlik ◽  
Jan Muzik ◽  
...  

Abstract Abstract 2852 Background: The chronic lymphocytic leukemia is the most frequent leukemia in the western world predominantly diagnosed in older population. It is still considered to be incurable disease, but the significant proportion of patients do not require any treatment during the course of disease. The outcome is influenced by many factors including leukemia biology, patient's status, complications based on immune dysfunction, treatment choice, supportive care and other factors. Clinical trials are focused on highly selected population in need of treatment. Population based data providing the full picture is extremely rare, especially with the treatment data. The presented population-based study provides the full picture of CLL population including the untreated as well treated cohorts in different age and gender subgroups. Methods: Using data from the nationwide, population-based Czech Cancer Registry (CCR) and Czech population life-tables provided by the Czech Statistical Office to the Human Mortality Database stratified by age, sex, and calendar time we characterized trends in incidence, mortality and prevalence for all pts diagnosed with CLL in Czech Republic 1979–2008 and relative survival for patients diagnosed in five calendar periods (1980–1984, 1985–1989, 1990–1994, 1995–1999, 2000–2003). All computations were performed using Stata 10.1™ software. Results: Altogether 13, 162 pts with CLL diagnosis have been reported to the CCR from 1979 till 2008. Median age 70 years at diagnosis remains unchanged during the whole period, the male/female ratio was 1.5: 1. The CLL incidence increased from 3.3 in 1979 to 5.6 per 100 thousands inhabitants in 2008. The therapy was administered in 54% of all patients, with the significant trend to decrease with calendar period from 60.4% for pts dg in 1980–84 period to 42.3% in 200019403 period. The treatment was administered in 70.2% of pts younger than 60 y compared to 50.1% of older than 60y with the trend to decreased number of treated pts during the time in both cohorts (from 78.5% to 55.3% for younger and from 55.4% to 38.7% for older cohort). The improvement in five years relative survival ratios (RSRs; 95% confidence interval) with calendar period was observed for all patients starting at 0.46 (0.39–0.53), 0.48 (0.42–0.55), 0.52 (0.46–0.59), 0.60 (0.54–0.66) to 0.69 (0.62–0.74). The 5 y RSRs was better for untreated patients compared to treated pts resp. with improvement in both cohorts from 0.53 (0.44–0–62) and 0.34 (0.25–0.45) resp. in 1980–4 period to 0.77 (0.70–0.84) and 0.51 (0.42–0.60) resp. in 2000–3 period. The same trend was observed for young and old, untreated and treated pts. The females have significant better outcome compared to males in all cohorts, young as well old, untreated as well treated. Conclusions: In this large population-based study with more than 13, 000 patients we confirmed the increased CLL incidence together with decreased need of treatment which can be explained by the higher number of patients diagnosed in early stages in the recent time. The patients without need of treatment have siginificantly better 5y RSRs compared to pts with need of treatment, the females have significantly better 5y RSRs compared to males consistently in all subgroups. The patient's survival was improved with calendar period for the whole CLL population as well for the younger and older, females and males, untreated as well treated cohort. Disclosures: No relevant conflicts of interest to declare.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sophie H. Bots ◽  
Klaske R. Siegersma ◽  
N. Charlotte Onland-Moret ◽  
Folkert W. Asselbergs ◽  
G. Aernout Somsen ◽  
...  

Abstract Background Despite the increasing availability of clinical data due to the digitalisation of healthcare systems, data often remain inaccessible due to the diversity of data collection systems. In the Netherlands, Cardiology Centers of the Netherlands (CCN) introduced “one-stop shop” diagnostic clinics for patients suspected of cardiac disease by their general practitioner. All CCN clinics use the same data collection system and standardised protocol, creating a large regular care database. This database can be used to describe referral practices, evaluate risk factors for cardiovascular disease (CVD) in important patient subgroups, and develop prediction models for use in daily care. Construction and content The current database contains data on all patients who underwent a cardiac workup in one of the 13 CCN clinics between 2007 and February 2018 (n = 109,151, 51.9% women). Data were pseudonymised and contain information on anthropometrics, cardiac symptoms, risk factors, comorbidities, cardiovascular and family history, standard blood laboratory measurements, transthoracic echocardiography, electrocardiography in rest and during exercise, and medication use. Clinical follow-up is based on medical need and consisted of either a repeat visit at CCN (43.8%) or referral for an external procedure in a hospital (16.5%). Passive follow-up via linkage to national mortality registers is available for 95% of the database. Utility and discussion The CCN database provides a strong base for research into historically underrepresented patient groups due to the large number of patients and the lack of in- and exclusion criteria. It also enables the development of artificial intelligence-based decision support tools. Its contemporary nature allows for comparison of daily care with the current guidelines and protocols. Missing data is an inherent limitation, as the cardiologist could deviate from standardised protocols when clinically indicated. Conclusion The CCN database offers the opportunity to conduct research in a unique population referred from the general practitioner to the cardiologist for diagnostic workup. This, in combination with its large size, the representation of historically underrepresented patient groups and contemporary nature makes it a valuable tool for expanding our knowledge of cardiovascular diseases. Trial registration: Not applicable.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii322-iii322
Author(s):  
Raoull Hoogendijk ◽  
Jasper van der Lugt ◽  
Dannis van Vuurden ◽  
Eelco Hoving ◽  
Leontien Kremer ◽  
...  

Abstract BACKGROUND Variation in survival of pediatric central nervous system (CNS) tumors is large between countries. Within Europe, the Netherlands had one of the worst reported survival rates of malignant CNS (mCNS) tumors during 2000–2007. METHODS Using the Netherlands Cancer Registry, we evaluated trends in incidence and survival of pediatric mCNS tumors (behavior /3, 5th digit in the morphology code) diagnosed between 1990–2017. RESULTS 839 newly-diagnosed mCNS tumor patients &lt;18 years were registered between 1990–2017. Incidence of mCNS tumors remained stable (average incidence rate, 21.6 per million person-years). However, an increased incidence of malignant gliomas, NOS was found (Estimated Annual Percentage Change (EAPC) 11.6% p&lt;0.001). This appears to be related to a registration shift between 1990–1999 and 2000–2009 as brainstem tumors increased (+25%, n=79) for astrocytomas and other gliomas but decreased (-31%, n=32) for unspecified intracranial and intraspinal neoplasms. Overall, 5-year observed survival (5Y-OS) of mCNS tumors increased from 51% in 1990–1999 to 61% in 2010–2017 (P-for-trend&lt;0.001). This increase was not constant over time, as 5Y-OS for the period 2000–2009 was 47%. The only significant decrease in survival was found for malignant astrocytomas and other gliomas with a 5Y-OS of 56% in 1990–1999 decreasing to 48% in 2010–2017 (P-for-trend&lt;0.001). CONCLUSION Between 1990–2017 incidence of mCNS tumors in the Netherlands remained stable and survival increased. However, a decrease in survival was seen for malignant astrocytomas and other gliomas, which is partially explained by the registration shift of brainstem tumors. The impact of this shift on survival for all mCNS tumors is subject to further research.


2018 ◽  
Vol 23 (suppl_1) ◽  
pp. e44-e45 ◽  
Author(s):  
Hana Mijovic ◽  
Yossef Al-Nasser ◽  
Ghada Al-Rawahi ◽  
Ashley Roberts

Abstract BACKGROUND Tuberculosis (TB) is a rare but potentially devastating infection among Canadian children. Accurate diagnosis and initiation of treatment are limited in part by the fact that it takes 2–6 weeks for culture results to be confirmed. Xpert MTB/RIF (Xpert) is a rapid, automated molecular assay that has been validated for diagnosing pulmonary but not extra-pulmonary TB in children. OBJECTIVES This was a retrospective study of children investigated for active TB at our facility in order to: 1.Outline demographic characteristics and describe clinical presentations of children diagnosed with active TB. 2.Compare performance of molecular testing (Xpert) to stain and Mycobacterium tuberculosis culture on pulmonary and extra-pulmonary specimens. DESIGN/METHODS We conducted a retrospective chart review of all paediatric patients investigated for active TB at our facility with stain, culture and molecular (Xpert) testing between January 2015 and August 2017. Due to a small number of patients, our data analysis was limited to narrative summary and descriptive statistics. RESULTS A total of 10 children were diagnosed with active TB, including 3 cases of pulmonary, 4 extra-pulmonary and 3 disseminated disease. Age range at diagnosis was 2 months to 16 years, with 3 children younger than 1 year. Most children contracted TB while travelling to and/or being exposed to an index case from endemic areas, including East Asia/Western Pacific (5), South Asia (2) and Africa (1). All children were HIV negative. Time from symptom onset to TB diagnosis and treatment ranged from approximately 4 days to 5 months. Multi-drug resistant TB was confirmed in 1 child. Sadly, 1 child passed away from TB related complications. AFB stain was positive on at least one specimen in 4/10 cases, cultures were positive in 8/10 and molecular testing (Xpert) in 7/10 cases. Time to positive cultures ranged from 10 to 35 days, with an average of 19 days. All cases positive on Xpert were also culture positive. Xpert test diagnosed TB in 5/6 of extra-pulmonary specimens submitted, including pericardial fluid, lymph node tissues and cerebrospinal fluid. CONCLUSION Many paediatric TB patients at our facility are children who have traveled to/have contacts from TB endemic regions, emphasizing the need for obtaining thorough exposure and travel history. Culture and molecular testing demonstrated similar TB detection rates, albeit based on a small patient population. While cultures remain the most reliable diagnostic method, molecular testing may facilitate rapid diagnosis and treatment of pulmonary and extra-pulmonary paediatric TB in a non-endemic setting.


Mediscope ◽  
2018 ◽  
Vol 5 (2) ◽  
pp. 12-15
Author(s):  
MS Mallick

The aim of this descriptive type of study on diagnosed tuberculosis (TB) cases was to evaluate the incidence of TB in Narail district to have a situational analysis with the achievement of National TB Control Program. Related data were collected from the Civil Surgeon Office, Narail district. The data of 3 upazilas- Narail Sadar, Kalia, and Lohagora were used. The study period was from January 1, 2016 to December 31, 2016. The overall TB case rate was 156.6 per 100000 population and separately 170.9 per 100000 population in Narail Sadar, 142.2 per 100000 population in Kalia and 156.6 per 100000 population in Lohagora. Of the cases, 56.4% were male and 43.6% female. Overall, 89.7% of the TB cases had pulmonary TB and 10.3% had extra-pulmonary TB. The population with higher age had higher proportion having TB. The highest number of patients were referred for diagnosis by sastha sebika (nurse) and non-government field staff. The TB incidence in Narail district was considered to be lower than the National situation. However, it seems that TB will remain as a continuous concern for many years in future for the country as well as Narail district. The activities of National TB Control Program of the Government of Bangladesh needs to be strengthened further to combat TB in BangladeshMediscope Vol. 5, No. 2: Jul 2018, Page 12-15


2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Grażyna Wyszyńska-Pawelec ◽  
Michał Gontarz ◽  
Jan Zapała ◽  
Mariusz Szuta

The aim of this retrospective study of 56 patients with minor salivary gland tumours (MSGTs) of the upper aerodigestive tract is to present demographic features, distribution of tumours as well as methods and results of treatment performed in our institution over a 10-year period. Of 221 patients with salivary gland tumours, 56 patients with MSGT were selected. There were 36 female and 20 male patients aged from 8 to 81 years. Male-to-female ratio was 1 : 2 in the group of benign MSGT and 1 : 1.7 in the group of malignant tumours. The palate was the most frequent site of MSGT (45.6%), followed by buccal mucosa (19.3%). Of all MSGTs 63.2% were malignant, and 36.8% were benign. Adenoid cystic carcinoma was the most common neoplasm (31.6%), followed by pleomorphic adenoma (29.8%). Surgery was the method of choice in the treatment of patients with MSGT. Postoperative defects were reconstructed by prosthetic obturators, local flaps, and free radial forearm flap. Relative survival for patients with malignant MSGT was 88% at three years and 71.5% at five years. MSGTs are more frequent in females and predominantly affect the palate. Malignant MSGTs are more common than benign.


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