Malignant neoplasms of kidney, except renal pelvis

**Definition:** Cancer of the kidney amounts to 2% of the total human cancer burden, with approximately 190,000 new cases diagnosed each year. They occur in all world regions, with a preference for developed countries. Etiological factors include environmental carcinogens (tobacco smoking) and lifestyle factors, in particular obesity. Although renal tumours can be completely removed surgically, haematogeneous metastasis is frequent and may occur already at an early stage of the disease. The pattern of somatic mutations in kidney tumours has been extensively investigated and has become, in addition to histopathology, a major criterion for classification. Kidney tumours also occur in the setting of several inherited cancer syndromes, including von Hippel-Lindau disease.

**Exclusions:** - Malignant mesenchymal neoplasms - Malignant neoplasm metastasis in kidney or renal pelvis - Malignant neoplasms of renal pelvis - Malignant neoplasm of renal calyces