21st June 2023

Renal Cancer

Renal cancers are differentiated between those of the renal parenchyma (renal cell carcinoma), renal tract (urothelial carcinomas; formerly transitional cell carcinoma) as well as rarer entities including Wilms’ tumours and neuroblastoma. Increasingly, they present as an incidental finding on imaging for other reasons. They can present with flank/back pain, masses, haematuria, recurrent infections, and weight loss.

Surgery remains the mainstay of radical treatment for malignancies of the renal tract. There is no established role for neoadjuvant or adjuvant therapy for renal cell carcinomas, although this is the subject of clinical trials. There is a role for adjuvant chemotherapy for upper tract urothelial malignancies, as per bladder cancer. Management of metastatic disease includes immunotherapy as well as various tyrosine kinase inhibitors.

Questions within the part 1 exam pertaining to malignancies of the renal tract tend to focus on familial syndromes. As a more general tip, it would be wise to revise the more common familial cancer syndromes. For renal malignancies these syndromes include:

• Von Hippel-Lindau syndrome – Caused by a pathological mutation of the VHL gene. Results in clear cell carcinoma of the kidney as well as haemangioblastomas of the central nervous syndrome, phaeochromocytomas, inner-ear tumours and neuroendocrine tumours of the pancreas.
• Tuberous sclerosis – Caused by a pathological mutation of the TSC1 & TSC2 genes. Results in angiomyolipomas of the kidney and subependymal giant cell astrocytomas. Other manifestations include ungual/periungual fibromas, facial angiofibroma and hypomelanotic macules.
• PTEN hamartoma syndrome – Caused by a pathological mutation of the PTEN gene causing clear cell carcinomas as well as associations with breast and thyroid cancer. Patients exhibit macrocephaly, mucocutaneous papules, hamartomas, and lipomas.
• Birt-Hogg-Dube syndrome – Caused by a pathological mutation of the FLCN gene causing oncocytic and chromophobe renal cell carcinomas as well as lung cysts and fibrofolliculomas.