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Anatomic Location-Specific Screening for Adenocarcinoma in EMPD

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. 

Source: jaad.org/article/S0190-9622(24)02993-1/abstract