A 40-year-old woman with Cowden syndrome presents for routine gynecologic screening. Which gynecologic malignancy is she at the highest risk of developing?

• A Ovarian serous carcinoma
• B Vulvar melanoma
• C Cervical squamous cell carcinoma
• D Endometrial carcinoma ✓

Explanation

Cowden syndrome, caused by PTEN mutations, significantly increases the risk of endometrial carcinoma, with a lifetime risk of approximately 13 to 28 percent. It also raises the risk of breast and thyroid cancers. Ovarian serous carcinoma is not the primary gynecologic malignancy associated with this syndrome. Vulvar melanoma and cervical squamous cell carcinoma are not characteristic components of the Cowden syndrome tumor spectrum.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.