The Paget's disease of the nipple is characterized histologically by large pale cells with prominent nucleoli within the epidermis. Which of the following statements about its management is MOST accurate?

• A It is always associated with an underlying invasive carcinoma and mandates mastectomy
• B Nipple-sparing mastectomy is contraindicated because the nipple-areolar complex is diseased
• C When no underlying mass is detected on imaging, breast-conserving surgery with central lumpectomy followed by radiotherapy is an acceptable alternative to mastectomy ✓
• D Paget's cells are derived from lymphocytes migrating from underlying ductal carcinoma in situ

Explanation

Paget's disease is a malignant condition of the nipple epidermis and is associated with underlying DCIS in ~80% and invasive carcinoma in ~50% of cases; not all have invasive disease. When imaging reveals no mass lesion and disease appears localised, breast-conserving surgery (central segment resection including the nipple-areolar complex) followed by whole-breast irradiation is an oncologically acceptable option with outcomes equivalent to mastectomy in appropriately selected patients. Option A overstates the association with invasive carcinoma. Option B is incorrect in context — nipple-sparing is indeed contraindicated, but the question asks about the correct management not a contraindication statement. Option D is incorrect; Paget's cells are believed to arise from ductal carcinoma cells that migrate epidermotropically.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.