Histopathology of a cervical mass from a 36-year-old woman shows extremely well-differentiated glands lined by bland, cytologically benign-appearing epithelium infiltrating deeply into the cervical stroma with minimal stromal reaction. Which association is characteristically described with this tumour?

• A Lynch syndrome
• B Peutz-Jeghers syndrome ✓
• C Li-Fraumeni syndrome
• D Cowden syndrome

Explanation

The description is of minimal deviation adenocarcinoma, historically called adenoma malignum. Its deceptively benign glands make it a notorious diagnostic pitfall on biopsy, and it is classically associated with Peutz-Jeghers syndrome, particularly the STK11 mutation. Lynch syndrome is linked to endometrioid and colorectal malignancies rather than this variant, which kills option A. Most cases are HPV independent, unlike usual-type cervical adenocarcinoma.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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