19 - Wilms' tumor

Abstract

This chapter discusses the clinical features, signs and symptoms, pathology, and treatments of Wilms' Tumor. Wilms' tumor is the second most common malignant retroperitoneal tumor. It is the most common primary renal tumor of childhood. The tumor is derived from primitive metanephric blastema and is characterized by histopathologic diversity and is composed of persistent blastema, dysplastic tubules, and supporting mesenchyme or stroma. The incidences of congenital anomalies in patients with Wilms' tumor are genitourinary anomalies, congenital aniridia, congenital hemihypertrophy, musculoskeletal anomalies, and hamartomas. Initial signs and symptoms of the tumor are palpable mass in abdomen, hypertension, hematuria, obstipation, weight loss, urinary tract infection, diarrhea, and previous trauma. The major investigations made in the patients are physical examination, history, complete blood count, blood chemistries, assessment of coagulation factors, assessment of cardiac status, abdominal ultrasound, abdominal CT scan, chest radiograph, chest CT scan, skeletal scintigram, magnetic resonance imaging and/or CT scan of brain, and peripheral blood for chromosomal analysis. Recessive cancer genes (suppressor genes) play an important role in the pathogenesis of Wilms' tumor. The Wilms' tumor 1 (WT1) gene encodes a transcription factor that is important in normal kidney and gonadal development. The basic proposed treatments given to the patients of Wilms' tumor are: radiation therapy, surgery, and chemotherapy.