Preface

Wilms’ tumor (WT), also called nephroblastoma, is a rare kidney cancer that is usually diagnosed in children under the age of 6. WT arises from nephrogenic rests, which are undifferentiated embryonic tissues retained after birth. At molecular level, in a proportion of patients, WT has been shown to be the result of aberrations in WT1 gene, located on chromosome 11p13. In addition to being a risk factor for WT, germ line WT1 aberrations can cause renal and extrarenal developmental abnormalities and predispose to other malignancies. In the past two decades, there has been a considerable improvement in our understanding of WT and WT1. This book brings together recently uncovered basic and clinical aspects of the burgeoning WT and WT1 research under three sections: epidemiology and clinical aspects, biology, and WT1 gene aberrations in other malignancies.

Section I provides a comprehensive guide to the epidemiology, diagnosis, management, and treatment of WT. Chapter 1 describes the morphology and differential diagnosis of WT. It presents a clear view of the common histological components of WT. While stage and histological subtypes are well-known prognostic factors for WT, age at diagnosis is also an independent risk factor for recurrence. Chapter 2 elegantly summarizes the clinical relevance of age at presentation in WT management. Read more…..