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 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.

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 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.

Pediatric Renal Tumors: Practical Updates for the Pathologist

2005 ◽  
Vol 8 (3) ◽  
pp. 320-338 ◽  
Author(s):  
Elizabeth J. Perlman
Keyword(s):  
Molecular Analysis ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Regional Lymph Node ◽  
Clear Cell Sarcoma ◽  
Considerable Change ◽  
Stage I ◽  
Renal Tumors ◽  
Favorable Histology ◽  
Pathologic Classification

Pediatric renal tumors were targeted by the National Wilms Tumor Study Group for 4 decades with extraordinary success. Within this historic context, this review provides a summary of the new Children's Oncology Group renal tumor protocols that will be opening in the very near future, focusing on their pathologic requirements. All renal tumors must first be registered on the Renal Tumor Classification and Banking Protocol, followed by registration on 1 of 4 primary therapeutic protocols based on histology, stage, and molecular analysis. This requires prompt submission of samples for molecular analysis and central pathologic review. Changes in staging criteria include classification of all tumor spillage as stage III, and requirement of regional lymph node evaluation for eligibility for stage I Wilms tumors (WTs) weighing less than 550 g in infants younger than 24 months and for stage I clear cell sarcoma. Patients with unilateral favorable histology WT with loss of heterozygosity for chromosomes 1p and 16q will receive more aggressive chemotherapy at each stage. Patients with bilateral WT and patients with diffuse hyperplastic perilobar nephroblastomatosis will be eligible for a novel therapeutic protocol requiring pathologic classification based on response of tumor to previous therapy. Stage I anaplastic WT will be targeted with more aggressive chemotherapy than in the past. For the first time, pediatric renal cell carcinoma will be eligible for a cooperative group protocol. All rhabdoid tumors outside the central nervous system will be eligible for a single protocol. In conclusion, these new protocols bring considerable change in their overall organization, in eligibility, and in therapy.

scholarly journals Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

2021 ◽  
Vol 19 (8) ◽  
pp. 945-977
Author(s):  
Frank Balis ◽  
Daniel M. Green ◽  
Clarke Anderson ◽  
Shelly Cook ◽  
Jasreman Dhillon ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Renal Tumors ◽  
Nccn Guidelines ◽  
Favorable Histology ◽  
Tumor Focus ◽  
Appropriate Treatment ◽  
Pediatric Oncologist

The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.

919: Ureteral Extension in Childhood Renal Tumors: A Report From the National Wilms Tumor Study Group (NWTSG)

2007 ◽  
Vol 177 (4S) ◽  
pp. 305-305
Author(s):  
Shane Daley ◽  
Michael Ritchey ◽  
Robert Shamberger ◽  
Robert Sawin ◽  
Thomas Hamilton ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Renal Tumors ◽  
Study Group ◽  
Tumor Study ◽  
Tumor Study Group

Genetic Diversity of Basil (Ocimum spp. - Lamiaceae) Based on RAPD Markers and Volatile Oil Constituents

HortScience ◽  
1998 ◽  
Vol 33 (3) ◽  
pp. 492f-493
Author(s):  
Roberto F. Vieira ◽  
James E. Simon ◽  
Peter Goldsbrough ◽  
Antonio Figueira
Keyword(s):  
Genetic Diversity ◽  
Molecular Markers ◽  
Rapd Markers ◽  
Volatile Oil ◽  
Essential Oil Composition ◽  
Oil Composition ◽  
Traditional Medicines ◽  
The Tropics ◽  
High Degree ◽  
First Time

Essential oils extracted from basil (Ocimum spp.) by steam distillation are used to flavor foods, oral products, in fragrances, and in traditional medicines. The genus Ocimum contains around 30 species native to the tropics and subtropics, with some species naturalized and/or cultivated in temperate areas. Interand intraspecific hybridization have created significant confusion in the botanical systematics of this genus. Taxonomy of basil (O. basilicum) is also complicated by the existence of numerous varieties, cultivars, and chemotypes within the species that do not differ significantly in morphology. In this study we are using RAPD markers and volatile oil composition to characterize the genetic diversity among the most economically important Ocimum species. We hypothesize that the genetic similarity revealed by molecular markers will more accurately reflect the morphological and chemical differences in Ocimum than essential oil composition per se. Preliminary research using five Ocimum species, four undetermined species, and eight varieties of O. basilicum (a total of 19 accessions) generated 107 polymorphic fragments amplified with 19 primers. RAPDs are able to discriminate between Ocimum species, but show a high degree of similarity between O. basilicum varieties. The genetic distance between nine species and among 55 accessions within the species O. americanum, O. basilicum, O. campechianum, O. × citriodorum, O. gratissimum, O. kilimandscharium, O. minimum, O. selloi, and O. tenuiflorum will be analyzed by matrix of similarity and compared to the volatile oil profile. This research will for the first time apply molecular markers to characterize the genetic diversity of Ocimum associate with volatile oil constituent.

scholarly journals Deep learning for end-to-end kidney cancer diagnosis on multi-phase abdominal computed tomography

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Kwang-Hyun Uhm ◽  
Seung-Won Jung ◽  
Moon Hyung Choi ◽  
Hong-Kyu Shin ◽  
Jae-Ik Yoo ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Deep Learning ◽  
Kidney Cancer ◽  
Renal Tumor ◽  
Renal Tumors ◽  
Abdominal Computed Tomography ◽  
Wide Range ◽  
End To End ◽  
Multi Phase ◽  
Histologic Subtypes

AbstractIn 2020, it is estimated that 73,750 kidney cancer cases were diagnosed, and 14,830 people died from cancer in the United States. Preoperative multi-phase abdominal computed tomography (CT) is often used for detecting lesions and classifying histologic subtypes of renal tumor to avoid unnecessary biopsy or surgery. However, there exists inter-observer variability due to subtle differences in the imaging features of tumor subtypes, which makes decisions on treatment challenging. While deep learning has been recently applied to the automated diagnosis of renal tumor, classification of a wide range of subtype classes has not been sufficiently studied yet. In this paper, we propose an end-to-end deep learning model for the differential diagnosis of five major histologic subtypes of renal tumors including both benign and malignant tumors on multi-phase CT. Our model is a unified framework to simultaneously identify lesions and classify subtypes for the diagnosis without manual intervention. We trained and tested the model using CT data from 308 patients who underwent nephrectomy for renal tumors. The model achieved an area under the curve (AUC) of 0.889, and outperformed radiologists for most subtypes. We further validated the model on an independent dataset of 184 patients from The Cancer Imaging Archive (TCIA). The AUC for this dataset was 0.855, and the model performed comparably to the radiologists. These results indicate that our model can achieve similar or better diagnostic performance than radiologists in differentiating a wide range of renal tumors on multi-phase CT.

Accuracy of MRI in the Differential Diagnosis of Wilms Tumor

2021 ◽  
Vol 15 (8) ◽  
pp. 2057-2059
Author(s):  
Maham Ashraf ◽  
Aysha Anjum ◽  
Eisha Tahir ◽  
Amber Goraya ◽  
Rabia Aqeel
Keyword(s):  
Differential Diagnosis ◽  
Diagnostic Accuracy ◽  
Gold Standard ◽  
Wilms Tumor ◽  
Correct Diagnosis ◽  
Renal Tumors ◽  
Common Finding ◽  
Predictive Values ◽  
Female Ratio ◽  
Male Predominance

Background & Objective: Renal tumors are a common finding in diagnostic imaging; these lesions usually are solid or cystic, benign or malignant, and the correct diagnosis may be difficult. The current study aims at to determine the diagnostic accuracy of magnetic resonance imaging for the diagnosis of Wilms tumor taking histopathology as gold standard. Methodology: This cross-sectional study was carried at the Department of Radiology, The Children’s Hospital & Institute of Child Health Lahore over 6 months from March 2019 to September 2019. The study involved 125 children of both genders aged between 2 years to 14 years presenting with a neoplastic mass in the retroperitoneum on ultrasound abdomen during initial workup. These children were assessed on MRI for the diagnosis of Wilms tumor. Later the diagnosis was confirmed on histopathology which was taken as gold standard and the diagnosis of MRI was judged accordingly as true/false and positive/negative. A written informed consent was obtained from every patient. Results: The mean age of the children was 5.8±3.9 years having a male predominance with male to female ratio of 1.8:1. Wilms tumor was suspected in 13 (10.4%) children on MRI. The diagnosis of Wilms tumor was confirmed in 13 (10.4%) children on histopathology. MRI was found to be 76.9% sensitive, 97.3% specific and 95.2% accurate with positive and negative predictive values of 76.9% and 97.3% respectively. Conclusion: In the present study, MRI was found to be 95.2% accurate in the differential diagnosis of Wilms tumor in children presenting with retroperitoneal mass which along with its non-invasive and radiation free nature advocates the preferred use of MRI in the diagnostic evaluation of such children in future oncologic practice. Keywords: Retroperitoneal Tumor, Wilms Tumor, MRI, Diagnostic Accuracy

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