Article
Oncology
Danny Legge, Ling Li, Whei Moriarty, David Lee, Marianna Szemes, Asef Zahed, Leonidas Panousopoulos, Wan Yun Chung, Yara Aghabi, Jasmin Barratt, Richard Williams, Kathy Pritchard-Jones, Karim T. A. Malik, Sebastian Oltean, Keith W. Brown
Summary: The study revealed that epigenetic inactivation of ESRP2 disrupts the mesenchymal to epithelial transition in early kidney development, leading to the formation of Wilms tumour (WT). Reactivation of ESRP2 expression by DNA methyltransferase inhibition suppressed cellular proliferation in WT cell lines and inhibited tumor growth in vivo.
MOLECULAR ONCOLOGY
(2022)
Article
Pediatrics
Radu Ninel Balanescu, Alexandru Emil Baetu, Andreea Alecsandra Moga, Laura Balanescu
Summary: This study demonstrates the value of ultrasound in the diagnosis of Wilms' tumour, as it can detect various important information, although it cannot replace CT in certain aspects.
Review
Cell Biology
Gordan M. Vujanic, Ellen D'Hooghe, Christian Vokuhl, Paola Collini
Summary: ICCR develops datasets for histopathological reporting of various cancer types, but international reporting guidelines for Wilms' tumors have not been established yet. Different treatment approaches result in varied assessment methods and classification criteria followed by countries worldwide.
Review
Oncology
Tessa Banki, Jarno Drost, Marry M. M. van den Heuvel-Eibrink, Annelies M. C. Mavinkurve-Groothuis, Ronald R. R. de Krijger
Summary: This study reviewed the molecular changes in nephrogenic rests (NR), which are believed to be precursor lesions of Wilms tumors (WT), the most common malignant childhood renal tumors, from 1990 to 2022. The findings showed that two genes, WT1 and WTX, and two chromosomal regions, 11p13 and 11p15, were mutated or showed loss of imprinting in both NR and WT, suggesting their potential roles in early tumorigenesis.
Review
Oncology
Thomas J. Jackson, Herve J. Brisse, Kathy Pritchard-Jones, Kayo Nakata, Carlo Morosi, Takahara Oue, Sabine Irtan, Gordan Vujanic, Marry M. van den Heuvel-Eibrink, Norbert Graf, Tanzina Chowdhury
Summary: The International Society of Paediatric Oncology Renal Tumour Study Group advocates preoperative chemotherapy for children with Wilms tumour, while other countries often choose to perform biopsies on some children with renal tumors to determine specific treatment plans.
PEDIATRIC BLOOD & CANCER
(2022)
Review
Oncology
Janna A. Hol, Rosalyn Jewell, Tanzina Chowdhury, Catriona Duncan, Kayo Nakata, Takaharu Oue, Marion Gauthier-Villars, Annemieke S. Littooij, Yasuhiko Kaneko, Norbert Graf, Franck Bourdeaut, Marry M. van den Heuvel-Eibrink, Kathy Pritchard-Jones, Eamonn R. Maher, Christian P. Kratz, Marjolijn C. J. Jongmans
Summary: The updated Wilms tumour surveillance guidelines have been released for clinical geneticists, pediatricians, pediatric oncologists, and radiologists caring for children at risk of WT. These guidelines stress the importance of registering all patients with cancer predisposition syndromes in order to improve future tumor risk estimation and surveillance programs.
EUROPEAN JOURNAL OF CANCER
(2021)
Review
Oncology
Janna A. Hol, Illja J. Diets, Ronald R. de Krijger, Marry M. van den Heuvel-Eibrink, Marjolijn C. J. Jongmans, Roland P. Kuiper
Summary: TRIM28 has been identified as a predisposition gene for Wilms' tumour (WT), with germline pathogenic variants associated with epithelial WT. Loss of TRIM28 protein expression in tumour tissue is an effective strategy for identifying patients with TRIM28 variants. Further exploration of TRIM28-associated WT will help uncover the mechanisms underlying tumour development.
JOURNAL OF PATHOLOGY
(2021)
Article
Cell Biology
Felix K. F. Kommoss, Anne-Sophie Chong, Maria Apellaniz-Ruiz, Gulisa Turashvili, Kay J. Park, Krisztina Hanley, Elvis Terci Valera, Andreas von Deimling, Gordan Vujanic, W. Glenn McCluggage, William D. Foulkes
Summary: In this study, we comprehensively characterized a series of five tumours originally diagnosed as ovarian WT, and found that teratoma-associated tumours and pure ovarian WT exhibit diverging morphological and molecular features.
Article
Oncology
Raquel Davila Fajardo, Rhoikos Furtwaengler, Martine van Grotel, Harm van Tinteren, Claudia Pasqualini, Kathy Pritchard-Jones, Reem Al-Saadi, Beatriz de Camargo, Gema L. Ramirez Villar, Norbert Graf, Xavier Muracciole, Patrick Melchior, Daniel Saunders, Christian Rube, Marry M. van den Heuvel-eibrink, Geert O. Janssens, Arnauld C. Verschuur
Summary: This study aims to explore the outcomes of metastatic patients with completely necrotic local stage III Wilms tumours, in relation to the application of postoperative radiotherapy. The results indicated excellent 5-year event-free survival and overall survival rates in this patient category, suggesting that abdominal irradiation may not be necessary in first-line treatment for these patients.
Article
Oncology
Prakriti Roy, Sophie E. van Peer, Rana Dandis, Catriona Duncan, Joaquim Caetano de Aguirre-Neto, Arnauld Verschuur, Beatriz de Camargo, Henrike E. Karim-Kos, Luna Boschetti, Filippo Spreafico, Gema L. Ramirez-Villar, Norbert Graf, Harm van Tinteren, Kathy Pritchard-Jones, Marry M. van den Heuvel-Eibrink
Summary: The COVID-19 pandemic had a brief impact on the characteristics of paediatric renal tumour patients, leading to a decrease in newly diagnosed cases, an increase in disease severity, and larger tumour volumes. However, there were no delays in patient referral or treatment initiation.
Article
Immunology
Xiao-Mao Tian, Bin Xiang, Li-Ming Jin, Tao Mi, Jin-Kui Wang, Chenghao Zhanghuang, Zhao-Xia Zhang, Mei-Ling Chen, Qin-Lin Shi, Feng Liu, Tao Lin, Guang-Hui Wei
Summary: A novel immune signature has been established using high-throughput RNA sequencing and analysis of immune-related genes, which can predict prognosis and immunotherapy response in children with Wilms tumour (WT). This signature also reflects immune status and immune cell infiltration in the tumour microenvironment, and is significantly associated with immune checkpoint molecules.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Oncology
Alissa Groenendijk, Harm van Tinteren, Yilin Jiang, Ronald R. de Krijger, Gordan M. Vujanic, Jan Godzinski, Christian Rube, Jens-Peter Schenk, Carlo Morosi, Kathy Pritchard-Jones, Reem Al-Saadi, Sucheta J. Vaidya, Arnauld C. Verschuur, Gema L. Ramirez-Villar, Norbert Graf, Beatriz de Camargo, Jarno Drost, Daniela Perotti, Marry M. Van den Heuvel-Eibrink, Jesper Brok, Filippo Spreafico, Annelies M. C. Mavinkurve-Groothuis
Summary: This study retrospectively evaluated the rescue treatment and survival of patients with relapsed Wilms tumor (WT) after receiving vincristine and actinomycin-D treatment. The results showed that there was no significant difference in event-free survival and overall survival between patients treated with VAD regimens and those treated with more intensive regimens.
EUROPEAN JOURNAL OF CANCER
(2022)
Article
Oncology
Gordan M. Vujanic, Norbert Graf, Ellen D'Hooghe, Tanzina Chowdhury, Christian Vokuhl, Reem Al-Saadi, Kathy Pritchard-Jones, Patrick Melchior, Rhoikos Furtwaengler
Summary: This study retrospectively analyzed the outcomes in patients with stage III Wilms' tumor due to positive resection margins (RM). The results showed that patients without viable tumor had better local-relapse-free survival (RFS) and event-free survival (EFS) compared to patients with viable tumor.
INTERNATIONAL JOURNAL OF CANCER
(2023)
Article
Oncology
Tao Mi, Liming Jin, Zhaoxia Zhang, Jinkui Wang, Mujie Li, Chenghao Zhanghuang, Xiaojun Tan, Zhang Wang, Xiaomao Tian, Bin Xiang, Dawei He
Summary: This study aims to investigate the role of chemokines in Wilms tumours, and it found that 16 chemokines were differentially expressed in tumor and para-tumor samples. Furthermore, 7 chemokines were associated with survival. A predictive model based on chemokines was constructed, and it showed good prediction ability. The infiltration of T cells in Wilms tumor was found to be low, and the chemokine CX3CL1 was strongly correlated with T cells. The study also revealed DNA methylation differences between tumor and para-tumor samples, and CX3CL1 was hypermethylated in tumors with a negative correlation with mRNA expression.
FRONTIERS IN ONCOLOGY
(2022)
Article
Oncology
Marianna Szemes, Zsombor Melegh, Jacob Bellamy, Ji Hyun Park, Biyao Chen, Alexander Greenhough, Daniel Catchpoole, Karim Malik
Summary: Wilms' tumour (WT) is a common solid paediatric malignancy, with successful treatment in most cases, but poorer prognosis in cases with anaplastic histology. Studies have shown increased expression of MYCN in anaplastic WT, and its regulation of various genes and pathways. Further research on MYCN in WT could lead to potential targeted therapeutics for poor-prognosis cases.
Article
Oncology
Lesley Smith, Charles A. Stiller, Joanne F. Aitken, Lisa L. Hjalgrim, Tom Johannesen, Paivi Lahteenmaki, Martin G. McCabe, Robert Phillips, Kathy Pritchard-Jones, Eva Steliarova-Foucher, Jeanette F. Winther, Ryan R. Woods, Adam W. Glaser, Richard G. Feltbower
Summary: Despite significant temporal reductions in cancer mortality rates for all cancers and age groups in the six ICBP countries, the greatest improvements were seen in children aged 0-14 compared to adolescents and adults. This may be attributed to better care and treatment protocols for children.
INTERNATIONAL JOURNAL OF CANCER
(2022)
Review
Oncology
Thomas J. Jackson, Herve J. Brisse, Kathy Pritchard-Jones, Kayo Nakata, Carlo Morosi, Takahara Oue, Sabine Irtan, Gordan Vujanic, Marry M. van den Heuvel-Eibrink, Norbert Graf, Tanzina Chowdhury
Summary: The International Society of Paediatric Oncology Renal Tumour Study Group advocates preoperative chemotherapy for children with Wilms tumour, while other countries often choose to perform biopsies on some children with renal tumors to determine specific treatment plans.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Health Policy & Services
Georgia B. Black, Victoria J. Wood, Angus I. G. Ramsay, Cecilia Vindrola-Padros, Catherine Perry, Caroline S. Clarke, Claire Levermore, Kathy Pritchard-Jones, Axel Bex, Maxine G. B. Tran, David C. Shackley, John Hines, Muntzer M. Mughal, Naomi J. Fulop
Summary: This study aimed to understand the perceptions of loss and the impact of leadership and management on coping strategies during the centralization of cancer services in England. The findings revealed that staff members perceived a loss of surgical activity, skills, and experienced teams during the centralization process, which had long-term implications on their emotional well-being and motivation. Although leaders implemented instrumental measures to mitigate these losses, their patchy implementation and negative impacts on individuals hindered the effectiveness. Limited emotional support was perceived to be offered.
JOURNAL OF HEALTH SERVICES RESEARCH & POLICY
(2022)
Article
Oncology
Julia M. Gumy, Allison Silverstein, Erica C. Kaye, Miguela A. Caniza, Maysam R. Homsi, Kathy Pritchard-Jones, Jessica M. Bate
Summary: This study aims to understand global caregiver concerns about SARS-CoV-2 vaccination for children with cancer and provide guidance for healthcare providers. A survey was conducted and the findings revealed caregivers' primary concerns regarding side effects and vaccine safety. Logistics-related questions were of concern for caregivers in low- and middle-income countries. Efforts are needed to provide tailored information and reduce global inequities in accessing vaccines.
PEDIATRIC HEMATOLOGY AND ONCOLOGY
(2023)
Article
Economics
Caroline S. Clarke, Mariya Melnychuk, Angus I. G. Ramsay, Cecilia Vindrola-Padros, Claire Levermore, Ravi Barod, Axel Bex, John Hines, Muntzer M. Mughal, Kathy Pritchard-Jones, Maxine Tran, David C. Shackley, Stephen Morris, Naomi J. Fulop, Rachael M. Hunter
Summary: This study investigated the impact of reconfiguring specialist surgery services for four cancers in London on costs and outcomes. The results showed that prostate cancer surgery reconfiguration had the highest probability of being cost-effective.
APPLIED HEALTH ECONOMICS AND HEALTH POLICY
(2022)
Article
Oncology
Miguela A. Caniza, Maysam R. Homsi, Jessica Bate, Riyadi Adrizain, Tarek Ahmed, Sarah Alexander, Arpita Bhattacharyya, Jose Luis Copado-Gutierrez, Ivan Gutierrez, Yan Yin Lim, Lisa Morrissey, Gita Naidu, Vivian Paintsil, Nita Radhakrishnan, Sheena Mukkada, Robert Phillips, Kenneth A. Alexander, Kathy Pritchard-Jones
Summary: This study aimed to answer questions regarding the use of COVID-19 vaccines in pediatric patients with cancer and provide recommendations for healthcare professionals and the public. A 12-member international working group selected and answered 15 key questions using a modified Delphi method. The results showed a high level of agreement among the group members for the 24 recommendations.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Oncology
Gordan M. Vujanic, Norbert Graf, Ellen D'Hooghe, Tanzina Chowdhury, Christian Vokuhl, Reem Al-Saadi, Kathy Pritchard-Jones, Patrick Melchior, Rhoikos Furtwaengler
Summary: This study retrospectively analyzed the outcomes in patients with stage III Wilms' tumor due to positive resection margins (RM). The results showed that patients without viable tumor had better local-relapse-free survival (RFS) and event-free survival (EFS) compared to patients with viable tumor.
INTERNATIONAL JOURNAL OF CANCER
(2023)
Article
Oncology
Ellen D'Hooghe, Rhoikos Furtwaengler, Tanzina Chowdhury, Christian Vokuhl, Reem Al-Saadi, Kathy Pritchard-Jones, Norbert Graf, Gordan M. Vujanic
Summary: This study analyzed the outcomes of patients with stage I epithelial type (ET-WT) or stromal type WT (ST-WT) treated with preoperative chemotherapy. The results showed that patients with stage I ET-WT or ST-WT had significantly better event-free survival compared to patients with other intermediate-risk WT. The study suggests that omission of postoperative chemotherapy could be considered for low-risk ET-WT or ST-WT patients.
Article
Oncology
Daniel C. Moreira, Ibrahim Qaddoumi, Yichen Chen, Nickhill L. Bhakta, Guillermo L. M. Chantada, Victor M. A. Santana, Miguela A. Caniza, Meenakshi Devidas, Kathy Pritchard-Jones, Carlos Rodriguez-Galindo, Eric Bouffet, Sheena Mukkada
Summary: This study describes the disease course and management of COVID-19 infection in children with central nervous system tumors. The frequency of severe infection appears to be low, but severe cases and deaths do occur.
PEDIATRIC BLOOD & CANCER
(2023)
Biographical-Item
Oncology
Alan Craft, Kathy Pritchard-Jones, Bernward Zeller, Yaddanapudi Ravindranat, Purna Kurkure
PEDIATRIC BLOOD & CANCER
(2023)
Review
Urology & Nephrology
Daniela Perotti, Richard D. Williams, Jenny Wegert, Jack Brzezinski, Mariana Maschietto, Sara Ciceri, David Gisselsson, Samantha Gadd, Amy L. Walz, Rhoikos Furtwaengler, Jarno Drost, Reem Al-Saadi, Nicholas Evageliou, Saskia L. Gooskens, Andrew L. Hong, Andrew J. Murphy, Michael V. Ortiz, Maureen J. O'Sullivan, Elizabeth A. Mullen, Marry M. van den Heuvel-eibrink, Conrad V. Fernandez, Norbert Graf, Paul E. Grundy, James I. Geller, Jeffrey S. Dome, Elizabeth J. Perlman, Manfred Gessler, Vicki Huff, Kathy Pritchard-Jones
Summary: The study of Wilms tumour has significantly advanced our understanding of its biology and genetics, with the development of the 'two-hit' model of tumour development and the discovery of the genetic landscape of the tumour. Efforts have also been made to find prognostic biomarkers and develop preclinical models. However, there are still many unanswered questions, such as the molecular understanding of relapse and the origins of bilateral Wilms tumour.
NATURE REVIEWS UROLOGY
(2023)
Meeting Abstract
Oncology
Nicola Hughes, Ashley Ball-Gamble, Kathy Pritchard-Jones, Chris Carrigan, Emily Connearn, Richard Feltbower, Angela Polanco, Lorna Fern
PEDIATRIC BLOOD & CANCER
(2022)
Article
Oncology
Joaquim Caetano de Aguirre-Neto, Beatriz de Camargo, Harm van Tinteren, Christophe Bergeron, Jesper Brok, Gema Ramirez-Villar, Arnauld Verschuur, Rhoikos Furtwaengler, Lisa Howell, Daniel Saunders, Oystein Olsen, Aurore Coulomb, Christian Vokuhl, Jan Godzinski, Anne M. Smets, Gordan M. Vujanic, Marry M. van den Heuvel-Eibrink, Norbert Graf, Kathy Pritchard-Jones
Summary: International benchmarking of survival rates from Wilms tumor within the International Society of Pediatric Oncology 2001 database revealed significant differences among different countries/regions. Despite similar age and histologic risk group distribution, variations in tumor stage distribution led to significant differences in 5-year event-free survival (EFS) and overall survival (OS).
JCO GLOBAL ONCOLOGY
(2022)
Review
Oncology
Ajay Major, Monica Palese, Ebru Ermis, Anthony James, Milena Villarroel, Federico Antillon Klussmann, Laila Hessissen, Jennifer Geel, Muhammad Saghir Khan, Rashmi Dalvi, Michael Sullivan, Pamela Kearns, A. Lindsay Frazier, Kathy Pritchard-Jones, Akira Nakagawara, Carlos Rodriguez-Galindo, Samuel L. Volchenboum
Summary: The article explores the global landscape of pediatric oncology clinical research and highlights the variability in structure, history, and goals of international pediatric cancer clinical trial collaborative groups. It emphasizes the need for tailored approaches to foster collaboration between different groups and emphasizes the importance of grassroots approaches, twinning relationships, and implementation science in achieving the goals of the WHO Global Initiative for Childhood Cancer.
JCO GLOBAL ONCOLOGY
(2022)
Article
Oncology
Angela Polanco, Reem Al-Saadi, Suzanne Tugnait, Nicole Scobie, Kathy Pritchard-Jones
Summary: Patient and Public Involvement and Engagement (PPIE) play a crucial role in pediatric oncology research, with patients, parents, and professionals driving research priorities. Disparities in PPIE activities across Europe and lack of involvement in some regions highlight the need for a clear European-level definition and further exploration of roles and responsibilities in PPIE activities. Best practice examples from different countries provide a preliminary evidence base for designing a European PPIE strategy framework inclusive of patient and parent voices.