A 36-year-old woman presents with copious watery mucoid vaginal discharge. Cervical biopsy shows deeply infiltrating glands lined by bland, extremely well-differentiated mucinous epithelium with minimal atypia, initially misread as benign. She has mucocutaneous pigmented macules on her lips. This lesion is associated with which syndrome?

• A Lynch syndrome
• B Cowden syndrome
• C Peutz-Jeghers syndrome ✓
• D Von Hippel-Lindau disease

Explanation

Minimal deviation adenocarcinoma, historically called adenoma malignum, is a gastric-type mucinous cervical adenocarcinoma composed of deceptively benign-looking glands that infiltrate deeply. It presents with watery mucoid discharge and is classically associated with Peutz-Jeghers syndrome, suggested here by mucocutaneous pigmentation. Lynch syndrome predisposes to ordinary endometrioid adenocarcinomas of endometrium and colorectum, not this entity.

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.