Treatment of Cowden Syndrome in Renal Cell Carcinoma

Cowden syndrome is a hereditary condition associated with an elevated risk of various neoplasms. Renal cell carcinoma represents one of the visceral malignancies encountered in this context, presenting a distinct clinical challenge.

No study has reported the specific characteristics of renal cell cancer in patients with Cowden syndrome / PTEN hamartoma tumour syndrome (CS/PHTS). Clinical management is therefore guided by evidence from sporadic renal cell cancer, adapted to this hereditary setting.

Management centres on surgical intervention, with the operative approach selected according to the degree of disease progression. Drug therapy may also be incorporated, following established strategies for sporadic renal cell cancer. The complete decision algorithm — including the full range of surgical options, conditions for each, and the role of systemic therapy — is detailed in the structured protocol.