Two Clinical Cases of Li-Fraumeni Syndrome and Prostate Cancer: Genetic Counseling and Clinical-Surgical Management.

Abstract

The present article treats about Li-Fraumeni syndrome (LFS), a genetic disease with autosomal dominant transmission which is associated with an increased predisposition to suffer certain cancers. However, prostate cancer has not been recognized as one of the main neoplasms associated with LFS, and so its diagnosis is not included in LFS screening programs.

Urologists should make a prompt identification of patients or relatives at risk of suffering Li- Fraumeni syndrome, and referral to a genetic counseling unit. Prompt diagnosis and treatment of prostate cancer is important.

Given the aggressive nature of the condition in carriers of a PT53 gene mutation, radical surgery is the first option in localized disease. As this condition confers higher risk of developing secondary tumors, radiotherapy and chemotherapy should be avoided. There is no clear evidence regarding the usefulness of hormone therapy in these patients.

We consider that the recommendations set out in the article can help urologist in the management of patients diagnosed with LFS and suspected prostate cancer.