Wilms tumor is a rare kidney cancer that is usually diagnosed in children under the age of six. Wilms tumor is mostly the result of aberrations in WT1 gene, located on chromosome 11p13. In addition to being a risk factor for Wilms tumor, WT1 aberrations cause extra renal complications and other malignancies. This book brings together the basic and clinical aspects of Wilms tumor and WT1 research under three sections. Section I provides a comprehensive guide to the epidemiology, diagnostics, management, and treatment of Wilms tumor. Section II covers the biological aspects of Wims tumor and WT1. Section III focuses on the role of WT1 in cardiac development, prostate cancer, glioblastoma and minimal residual disease.

Contents

• Foreword
  Bengt Sandstedt.
• Preface
  Marry M. van den Heuvel-Eibrink.
• Contributors
• Section I. Epidemiology, Diagnostics, and Treatment
  • 1. Wilms’ Tumour – Histology and Differential Diagnosis
    Sergey D. Popov, Neil J. Sebire, and Gordan M. Vujanic.
    • Introduction
    • Gross appearance
    • Histological features
    • Handling of the nephrectomy specimen
    • Nephrogenic rests and nephroblastomatosis
    • Differential diagnosis
    • Conclusion
    • Conflict of Interest
    • Acknowledgement
    • References
  • 2. The Clinical Relevance of Age at Presentation in Nephroblastoma
    Saskia L. Gooskens, Heidi Segers, Kathy Pritchard-Jones, Norbert Graf, Jeffrey S. Dome, and Marry M. van den Heuvel-Eibrink.
    • Introduction
    • The clinical relevance of age
    • Age in correlation with other prognostic factors
    • Conflict of Interest
    • Acknowledgment
    • References
  • 3. Histopathological and Molecular Characteristics of Wilms Tumor
    Gulden Diniz.
    • Introduction
    • Pathogenesis of WT
    • Common genetic abnormalities in WT
    • Histopathological features of WT
    • Clues for differential diagnosis of WT
    • Conclusion
    • Conflict of Interests
    • References
  • 4. Wilms Tumor and Its Management in a Surgical Aspect
    Basak Erginel.
    • Introduction
    • Clinical presentation
    • Diagnosis
    • Staging
    • Surgical management
    • Recurrent disease
    • Conclusion
    • Conflict of Interest
    • Acknowledgment
    • References
  • 5. Management of Bilateral Wilms Tumours
    Alastair J. W. Millar, Sharon Cox, and Alan Davidson.
    • Introduction
    • Predisposing factors
    • Investigation
    • Management
    • Prognosis
    • Conclusion
    • Conflict of Interest
    • References
  • 6. Extrarenal Wilms’ Tumor: Challenges in Diagnosis, Embryology, Treatment and Prognosis
    Reza Shojaeian, Mehran Hiradfar, Parisa Saeedi Sharifabad, and Nona Zabolinejad.
    • Introduction
    • Embryogenesis
    • Pathology
    • Clinical presentation and diagnosis
    • Treatment
    • Conclusion
    • Conflict of Interests
    • Acknowledgment
    • References
  • 7. Neoadjuvant Transcatheter Arterial Chemoembolization and Systemic Chemotherapy for the Treatment of Wilms Tumor
    Min-Ju Li, Da-Xing Tang, Shan Xu, Yong Huang, De-Hua Wu, Jin-Hu Wang, Can Lai, Hong-Feng Tang, and Qiang Shu.
    • Introduction
    • Patients and methods
    • Response to treatment
    • Discussion
    • Conclusion
    • Conflict of Interest
    • Acknowledgments
    • References
  • 8. Dendritic Cell-Based Cancer Immunotherapy Targeting Wilms’ Tumor 1 for Pediatric Cancer
    Shigetaka Shimodaira, Koichi Hirabayashi, Ryu Yanagisawa, Yumiko Higuchi, Kenji Sano, and Tomonobu Koizumi.
    • Introduction
    • Manufacture of a DC vaccine
    • DC vaccine release criteria
    • DC vaccine study
    • Case report
    • Immune monitoring with tetramer analysis
    • DC vaccination technology for pediatric patients
    • Conclusion
    • Conflicts of Interest
    • Acknowledgments
    • References
  • 9. Chronic Kidney Disease in Wilms Tumour Survivors – What Do We Know Today?
    Joanna Stefanowicz, Mateusz Kosiak, Wojciech Kosiak, and Beata S. Lipska-Ziętkiewicz.
    • Introduction
    • End-stage renal disease in WT
    • The function of solitary kidney in WTS
    • Chronic kidney disease in WTS
    • Conclusion
    • Conflicts of Interest
    • References
• Section II. Biology
  • 10. Gene Expression in Wilms Tumor: Disturbance of the Wnt Signaling Pathway and MicroRNA Biogenesis
    Dirce Maria Carraro, Rodrigo F. Ramalho, and Mariana Maschietto.
    • Introduction
    • Morphogenetic process of kidney development
    • Wnt signaling pathway
    • Wilms tumor and mutation repertoire
    • Conclusion
    • Conflict of Interest
    • Acknowledgment
    • References
  • 11. Transcriptional Regulation of the Human Thromboxane A₂ Receptor Gene by Wilms’ Tumour (WT)1
    B. Therese Kinsella.
    • Introduction
    • The role of thromboxane in cancer
    • Factors determining transcriptional regulation of TPα in platelet progenitor megakaryoblastic cells
    • Role of Wilms’ tumour 1 in regulating TPα expression in prostate and breast cancer
    • Conclusions and future perspectives
    • Abbreviations
    • Conflict of Interest
    • Acknowledgements
    • References
  • 12. The Inflammatory Microenvironment in Wilms Tumors
    Paramahamsa Maturu.
    • Introduction
    • What is Wilms tumor and what are the various components of Wilms tumor?
    • Types of Wilms tumors
    • What is known about kidney cancer and inflammation?
    • Molecular links between WT and inflammation
    • Inflammatory mediators
    • Other markers expressed in WTs
    • Cross talk between immune cells and other markers in WT microenvironment
    • Possible mechanism/mechanisms responsible for COX-2 pathway activation
    • Possible therapeutic targets in Wilms tumors
    • Conclusions
    • Conflict of Interests
    • Acknowledgment
    • References
• Section III. WT1 Gene Aberrations in Other Malignancies
  • 13. WT1 in Cardiac Development and Disease
    Sjoerd N. Duim, Marie-José Goumans, and Boudewijn P. T. Kruithof.
    • Introduction
    • WT1 in cardiac development
    • WT1 in the infarcted heart
    • The role of WT1 in endothelial cells
    • Clinical perspective
    • Conflict of Interest
    • Acknowledgment
    • References
  • 14. Functional Role of WT1 in Prostate Cancer
    Gail C. Fraizer, Kurtis Eisermann, Sony Pandey, Adina Brett-Morris, Anton Bazarov, Sarah Nock, Nirmala Ghimirey, and Steven J. Kuerbitz.
    • Introduction
    • Developmental expression of WT1
    • WT1 expression in non-Wilms cancer
    • Expression and potential role of WT1 in PC
    • WT1 may contribute to tumor angiogenesis via regulation of VEGF
    • Conclusion
    • Conflict of Interest
    • Acknowledgments
    • References
  • 15. Functional Roles of Wilms’ Tumor 1 (WT1) in Malignant Brain Tumors
    Noriyuki Kijima, Naoya Hashimoto, Yasuyoshi Chiba, Yasunori Fujimoto, Haruo Sugiyama, and Toshiki Yoshimine.
    • Introduction
    • WT1 expression in malignant brain tumors
    • Functional roles of WT1 in glioblastoma pathogenesis
    • Immunotherapy targeting WT1 peptide for malignant glioma
    • Conflict of Interest
    • Acknowledgment
    • References
  • 16. Wilms’ Tumor Gene (WT1) Expression and Minimal Residual Disease in Acute Myeloid Leukemia
    Giovanni Rossi, Maria Marta Minervini, Angelo Michele Carella, Lorella Melillo, and Nicola Cascavilla.
    • Introduction
    • WT1 as a minimal residual disease marker after conventional chemotherapy
    • WT1 as minimal residual disease marker in allogeneic stem cell transplantation
    • Conclusions
    • Conflict of Interest
    • Acknowledgment
    • References

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