scholarly journals Characteristics and outcome of children with renal tumors in the Netherlands: The first five-year’s experience of national centralization

PLoS ONE ◽  
2022 ◽  
Vol 17 (1) ◽  
pp. e0261729
Author(s):  
Prakriti Roy ◽  
Sophie E. van Peer ◽  
Martin M. de Witte ◽  
Godelieve A. M. Tytgat ◽  
Henrike E. Karim-Kos ◽  
...  
Keyword(s):  
The Netherlands ◽  
Pediatric Oncology ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Survival Rates ◽  
Renal Tumors ◽  
Childhood Malignancies ◽  
Netherlands Cancer Registry ◽  
Central Pathology ◽  
Centralized Care

Around 6% of all childhood malignancies represent renal tumors, of which a majority includes Wilms tumor (WT). Although survival rates have improved over the last decades, specific patients are still at risk for adverse outcome. In the Netherlands, since 2015, pediatric oncology care for renal tumors has been centralized in the Princess Máxima Center for Pediatric Oncology. Here, we describe experiences of the first 5 years of centralized care and explore whether this influences the epidemiological landscape by comparing data with the Netherlands Cancer Registry (NCR). We identified all patients <19 years with a renal mass diagnosed between 01-01-2015 and 31-12-2019 in the Princess Máxima Center. Epidemiology, characteristics and management were analyzed. We identified 164 patients (including 1 patient who refused consent for registration), in our center with a suspicion of a renal tumor. The remaining 163 cases included WT (n = 118)/cystic partially differentiated nephroblastoma (n = 2)/nephrogenic rests only (n = 6) and non-WT (n = 37). In this period, the NCR included 138 children, 1 17-year-old patient was not referred to the Princess Máxima Center. Central radiology review (before starting treatment) was performed in 121/163 patients, and central pathology review in 148/152 patients that underwent surgery. Treatment stratification, according to SIOP/EpSSG protocols was pursued based on multidisciplinary consensus. Preoperative chemotherapy was administered in 133 patients, whereas 19 patients underwent upfront surgery. Surgery was performed in 152 patients, and from 133 biomaterial was stored. Centralization of care for children with renal tumors led to referral of all but 1 new renal tumor cases in the Netherlands, and leads to referral of very rare subtypes not registered in the NCR, that benefit from high quality diagnostics and multidisciplinary decision making. National centralization of care led to enhanced development of molecular diagnostics and other innovation-based treatments for the future.

scholarly journals EPID-18. TRENDS IN INCIDENCE AND SURVIVAL OF MALIGNANT PEDIATRIC CENTRAL NERVOUS SYSTEM TUMORS IN THE NETHERLANDS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii322-iii322
Author(s):  
Raoull Hoogendijk ◽  
Jasper van der Lugt ◽  
Dannis van Vuurden ◽  
Eelco Hoving ◽  
Leontien Kremer ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
The Netherlands ◽  
Malignant Gliomas ◽  
Survival Rates ◽  
Tumor Patients ◽  
Netherlands Cancer Registry ◽  
Malignant Astrocytomas ◽  
Brainstem Tumors ◽  
The Impact

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.

scholarly journals Rising incidence, no change in survival and decreasing mortality from thyroid cancer in The Netherlands since 1989

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 ◽  
...  
Keyword(s):  
Thyroid Cancer ◽  
The Netherlands ◽  
Survival Rates ◽  
Age Groups ◽  
Relative Survival ◽  
Incidence Rates ◽  
Mortality Data ◽  
Netherlands Cancer Registry ◽  
Rising Incidence ◽  
Tumour Node

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.

scholarly journals Challenges on participation in a cooperative group of childhood renal tumors in Brasil

2020 ◽  
Vol 66 (3) ◽  
pp. 284-289
Author(s):  
Lucian S. Viana ◽  
Neimar de Paula Silva ◽  
Nathalie V. Balmant ◽  
Paulo A. Faria ◽  
Marceli O. Santos ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Cancer Registries ◽  
Geographic Region ◽  
Renal Tumors ◽  
Trial Participation ◽  
Cooperative Group ◽  
Cooperative Groups ◽  
Accrual Rate

SUMMARY OBJECTIVE Children with renal tumors included in clinical trials have significantly better outcomes. In Brasil, the enrollment of patients in clinical trials remains challenging. Here we aimed to describe participation accrual in the Brazilian Wilms Tumor Study Group (BWTSG) and to identify barriers to trial registration of children with renal tumors. METHODS We determined the numbers of renal tumor diagnoses in 105 hospital-based cancer registries from 2001-2009. We then compared these totals with the numbers of renal tumor cases registered in the BWTSG from the same hospitals during the same time period. We also invited members of the Brazilian Pediatric Oncology Society to complete a 5-point Likert-type scale questionnaire regarding their opinions of the importance of participation in cooperative group trials. RESULTS The accrual rate of patient participation per hospital varied from 25% to 76%, and was highest in the South region. The accrual rate of hospital participation also varied according to the region (20-31%) and was highest in the Southeast region. For the questionnaire regarding the importance of participation in cooperative groups, the responses showed an agreement of >75% on 10 of the 13 statements. CONCLUSION Our results demonstrated low accrual of participation in a cooperative group trial in Brasil. We identified variations in registration rates according to geographic region and hospital, which may help targeted efforts to increase registration rates. The survey responses demonstrated that colleagues understand the importance of trial participation.

Oncology

2020 ◽  
pp. 203-213
Author(s):  
Roy L. Kao ◽  
Maritza E. Ruiz ◽  
Moran Gotesman ◽  
James H. Ch’ng
Keyword(s):  
Pediatric Oncology ◽  
Hematopoietic Cell Transplantation ◽  
Wilms Tumor ◽  
Tumor Lysis Syndrome ◽  
Survival Rates ◽  
Germ Cell Tumors ◽  
Cancer Predisposition Syndrome ◽  
Pediatric Cancers ◽  
Questions And Answers ◽  
Board Review

Pediatric oncology focuses on the diagnosis and management of cancer in infants, children, and adolescents. Leukemias, lymphomas, and other hematological malignancies are the most common group of pediatric cancers, followed by brain tumors. Other important pediatric cancers include Wilms tumor, neuroblastoma, sarcomas, histiocytosis, and germ cell tumors. Certain cancers can present with oncologic emergencies, such as tumor lysis syndrome or spinal cord compression, or as part of a cancer predisposition syndrome. With increased survival rates, short- and long-term complications of chemotherapy, radiation, surgery, and hematopoietic cell transplantation are also prevalent and important to recognize. This chapter reviews these key concepts in pediatric oncology using board review–style questions and answers.

scholarly journals Childhood Renal Tumor: A Report from a Chinese Children’s Cancer Group

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Jiaoyang Cai ◽  
Ci Pan ◽  
Qin Lu ◽  
Jie Yan ◽  
Xiuli Ju ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Renal Tumor ◽  
Survival Rates ◽  
Renal Tumors ◽  
Group Model ◽  
Cooperative Group ◽  
Event Free Survival ◽  
Female Ratio ◽  
Free Survival ◽  
Favorable Histology

Here we investigated the establishment of multicenter cooperative treatment groups in China, as well as radiotherapy compliance and effectiveness among children with renal tumors. Medical records were reviewed for 316 children with renal tumors diagnosed by a multicenter cooperative group from 14 hospitals in China from 1998 to 2012. Median patient age was 29.5 months (range, 2–173 months old), and male-to-female ratio was 1.4 : 1. After a median follow-up of 22 months (range, 1–177 months), five-year event-free survival rates were 72% overall; 76.1% for favorable histology (251 cases); 59% for unfavorable histology (27 cases); and 91%, 75%, 71%, 53%, and 48.5%, respectively for Stages I, II, III, IV, and V. Following standardized criteria, radiation therapy was indicated for 153 patients, among whom five-year event-free survival was 72.8% for the 95 who received radiation and 24% for the 58 patients who did not. Our results are reasonable but can be further improved and show the feasibility of a multicenter cooperative group model for childhood renal tumor treatment in China. Radiation therapy is important for stage III and IV patients but remains difficult to implement in some parts of China. Government management departments and medical professionals must pay attention to this situation. This clinical trial is registered with ChiCTR-PRCH-14004372.

scholarly journals A case of Wilms tumor with right ventricular extension of tumor thrombus

2017 ◽  
Vol 4 (1) ◽  
pp. 60
Author(s):  
Mustafa Çakan ◽  
Ayşe Gülnur Tokuç ◽  
Kıvılcım Karadeniz Cerit ◽  
Koray Ak ◽  
Rabia Ergelen
Keyword(s):  
Inferior Vena Cava ◽  
Right Ventricle ◽  
Right Atrium ◽  
Tumor Thrombus ◽  
Inferior Vena ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Vena Cava ◽  
Renal Tumors ◽  
Tumor Extension

Primary renal tumors comprise 6% of all childhood cancers. Wilms tumor is the most common primary renal tumor in pediatric age group and the peak age of diagnosis is 3-4 years. In 10% of cases tumor extension into hepatic vein and inferior vena cava can be seen. But tumor extension into whole inferior vena cava, right atrium and right ventricle is only seen in less than 1% of patients. A 2-year-old girl was admitted to the hospital because of abdominal distension that was noticed by the parents two weeks ago. Imaging studies revealed that she had a mass at the right renal lodge which was favoring to Wilms tumor and on thorax tomography tumor thrombus was seen in the whole inferior vena cava, right atrium and right ventricle. Neoadjuvant chemotherapy was given for 7 weeks. On the 8th week of diagnosis, under cardiopulmonary bypass, surgical operation by pediatric and cardiovascular surgery teams for primary renal tumor and for cavo-atrial tumor thrombus was performed. Pathological examination of the mass was reported as stage 3 diffuse anaplastic Wilms tumor. The patient completed 24 weeks of chemotherapy protocol and she is being followed for 15 months without any morbidity. We present our case to emphasize the importance of multidisciplinary approach in Wilms tumor with cardiac extension.

A Composite Renal Tumor: Metanephric Adenofibroma, Wilms Tumor, and Renal Cell Carcinoma: A Missing Link?

2012 ◽  
Vol 15 (1) ◽  
pp. 65-70 ◽  
Author(s):  
Maria Laura Galluzzo ◽  
Maria T. Garcia de Davila ◽  
Gordan M. Vujanić
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Papillary Renal Cell Carcinoma ◽  
Renal Tumors ◽  
Metanephric Adenoma ◽  
Missing Link ◽  
Additional Support

A coexistence of different renal tumors has rarely been reported. The most commonly described association is of Wilms tumor and renal cell carcinoma. Metanephric adenofibroma has also been associated with Wilms tumor or papillary renal cell carcinoma. Another reported association is metanephric adenoma and papillary renal cell carcinoma with sarcomatoid dedifferentiation. Herein we describe a complex renal tumor containing areas of metanephric adenofibroma, Wilms tumor, and undifferentiated renal cell carcinoma in a previously healthy 18-year-old boy. The tumor showed histologic and immunohistochemical features of these 3 different tumors, offering additional support to the view that these 3 tumors are related.

scholarly journals Intra-tumor genetic heterogeneity in Wilms tumor samples

2019 ◽  
Vol 65 (12) ◽  
pp. 1496-1501 ◽  
Author(s):  
Bruna M de Sá Pereira ◽  
Rafaela Montalvão de Azevedo ◽  
Joaquim Caetano de Aguirre Neto ◽  
Clarice Franco Menezes ◽  
Karla Emília Rodrigues ◽  
...  
Keyword(s):  
Molecular Markers ◽  
Genetic Heterogeneity ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Renal Tumors ◽  
Childhood Cancers ◽  
Tumor Group ◽  
Somatic Alterations ◽  
Low Prevalence ◽  
High Degree

SUMMARY Childhood renal tumors account for ~7% of all childhood cancers, and most cases are embryonic Wilms’ tumors (WT). Children with WT are usually treated by either COG or SIOP. The later treats the children using preoperative chemotherapy, but both have around 90% of overall survival in five years. WT is a genetically heterogeneous group with a low prevalence of known somatic alterations. Only around 30% of the cases present mutation in known genes, and there is a relatively high degree of intra-tumor genetic heterogeneity (ITGH). Besides potentially having an impact on the clinical outcome of patients, ITGH may interfere with the search for molecular markers that are prospectively being tested by COG and SIOP. In this review, we present the proposal of the current UMBRELLA SIOP Study 2017/Brazilian Renal Tumor Group that requires the multi-sampling collection of each tumor to better evaluate possible molecular markers, as well as to understand WT biology

scholarly journals Survival rates of children and young adolescents with CNS tumors improved in the Netherlands since 1990: A population-based study

2021 ◽  
Author(s):  
Raoull Hoogendijk ◽  
Jasper van der Lugt ◽  
Dannis van Vuurden ◽  
Leontien Kremer ◽  
Pieter Wesseling ◽  
...  
Keyword(s):  
The Netherlands ◽  
Malignant Tumors ◽  
Survival Rates ◽  
Population Based ◽  
Cns Tumors ◽  
Mortality Trends ◽  
Netherlands Cancer Registry ◽  
Incidence And Mortality ◽  
Pilocytic Astrocytomas ◽  
Over Time

Abstract Introduction Survival of children with central nervous system (CNS) tumors varies largely between countries. For the Netherlands, detailed population-based estimation of incidence, survival and mortality of pediatric CNS tumors are lacking but are needed to evaluate progress. Methods All CNS tumors diagnosed in patients &lt;18 years during 1990-2017 were selected from the Netherlands Cancer Registry. Other than pilocytic astrocytomas, non-malignant tumors were included since 2000. Incidence and mortality trends were evaluated by Average Annual Percentage Change (AAPC). Changes over time in Five-year Observed Survival (5-year OS) were evaluated by Poisson regression models adjusted for follow-up time. Results Between 1990 and 2017, 2057 children were diagnosed with a malignant CNS tumor and 885 with a pilocytic astrocytoma. During 2000-17, 695 children were diagnosed with other non-malignant CNS tumors. Incidence rates of malignant tumors remained stable, while pilocytic astrocytomas and other non-malignant tumors increased by 2.0% and 2.4% per year, respectively. 5-year OS rates improved for all groups; however, improvement for malignant tumors was not constant over time. The contribution of malignant tumors located at the optic nerve tumors was 1% in 2000-09. However, shifting from pilocytic astrocytomas, increased to 6% in 2010-17, impacting survival outcomes for malignant tumors. Conclusion Survival rates of CNS tumors improved over time, but was not accompanied by a decreasing mortality rate. The observed temporary survival deterioration for malignant tumors appears to be related to changes in diagnostics and registration practices. Whether differences in treatment regimens contribute to this temporary decline in survival needs to be verified.

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