DOI: https://doi.org/10.36347/sjmcr.2026.v14i04.061

Pages: 844-847

PTEN hamartoma tumor syndrome (PHTS) is a spectrum of disorders caused by the PTEN mutation that include Cowden syndrome (CS), Bannayan-Riley-Ruvalcaba syndrome (BRRS), PTEN Related Proteus-like syndrome, adult Lhermitte-Duclos disease (LDD), and autism spectrum disorders with macrocephaly. In female patients, this syndrome is associated with up to a 50% lifetime risk of developing breast cancer, and current National Comprehensive Cancer Network (NCCN) Guidelines state that depending on the personal/family history, the option of risk-reducing bilateral mastectomy should be discussed with patients. However, there is little current literature regarding expected mammary histopathologic findings in these patients. Here, we present the histopathologic findings in a patient with Cowden Syndrome undergoing bilateral prophylactic mastectomy. Numerous benign lesions are seen with no evidence of malignancy, raising the question of a need for more rigorous risk assessment and clearer guidelines regarding management of these patients.