The Extramammary Paget’s disease (EMPD) was associated with underlying internal adenocarcinoma, known as secondary EMPD, though differences by an anatomic subtype and screening implications remained unclear.
Researchers conducted a retrospective study to determine the rates of secondary EMPD and the types of adenocarcinomas associated with EMPD anatomic subtype and to propose a screening algorithm for underlying the adenocarcinoma.
They performed a systematic literature review of EMPD from January 1990 to November 2022; 122 studies met the inclusion criteria. A multidisciplinary expert panel reviewed the recommendation statements on the adenocarcinoma screening.
The results showed that perianal EMPD was associated with a high rate of underlying adenocarcinoma (25%, primarily colorectal) compared with penoscrotal and vulvar EMPD (6% each, primarily of genitourinary origin). Thorough screening in perianal EMPD includes a colonoscopy, urine cytology, and computed tomography (CT) of the chest, abdomen, and pelvis. Cost-conscious screening tests in low-risk penoscrotal disease include urine cytology, heme-occult test, and prostate-specific antigen test (especially if under 70 years of age), additionally urine cytology, and mammography were recommended for low-risk vulvar EMPD, with high-risk features that may warrant more sensitive organ-specific testing.
Investigators concluded that screening for underlying the adenocarcinoma in EMPD should be guided by an anatomic location.