Wilms’ tumour

Wilms’ tumour

See also Chapter 17 . This is the most common tumour of childhood, account - ing for 5% of all childhood cancers. They are bilateral in 5% of cases and familial in 1%. The tumour has mixed elements derived from the embryonic nephrogenic tissue, namely blas - temal or undi ff erentiated tissue, epithelial tubules and stroma. The typical presentation is a child aged between 1 and 4 years of either gender with a large, palpable abdominal mass that may cross the midline. It may also be associated with haema - turia, hypertension, fever and weight loss. Pain is relatively uncommon. The large tumour can rupture and present as an acute abdomen. Other causes of renal masses include neuro - blastoma, congenital mesoblastic nephroma, RCC, clear-cell sarcoma and rhabdoid tumour. US can confirm the r enal origin and solid nature of the - mass. Further definitive imaging with either CECT or MRI is necessary to stage the disease. Up to 13% of patients hav e bilateral tumours. The tumours usually infiltrate the kidne ys and normal renal parenchyma is compressed at the periphery around the tumour ( claw sign ). A CT of the chest should be obtained as the lung is the most common site of distant metastasis. Current treatment is nephrectomy with pre- or postoperative chemotherapy . Both regimes have a comparable survival of ~90%. Tumours of the kidney and ureters /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF Brierley JD, Gospodarowicz MK, Wittekind C (eds). TNM classification of malignant tumours , 8th edn. Oxford: Wiley , 2016. Available from https://www .uicc.org/8th-edition-uicc-tnm-classification-malig - nant-tumors-published/. Khan F , Ahmed K, Lee N et al . Management of ureteropelvic junction obstruction in adults. Nature Rev Urol 2014; 11 (11): 629–38. Moore EE, Shackford SR, Pachter HL et al . Organ injury scaling: spleen, liver, and kidney . J Trauma 1989; 29 (12): 1664–6.

Rule out urothelial malignancy in high-risk adults (chronic smokers, occupational exposure, older age) with microscopic haematuria Nephron-sparing surgery should be considered in small renal masses to preserve renal function, more so in patients with compromised renal function PNSs are found in up to 30% and IVC tumour thrombus in 5–10% of patients with RCC Targeted therapy and immunotherapy have improved survival in metastatic RCC Wilms’ tumour is the most common renal tumour in children <15 years old and should be treated in a multidisciplinary setting