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Recommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic
Nour Kibbi1, Joshua L Owen2, Brandon Worley3
1Department of Dermatology, Stanford University School of Medicine, Palo Alto, California.
Journal of the American Academy of Dermatology
|October 14, 2024
Summary
Extramammary Paget's disease (EMPD) screening for adenocarcinoma should be guided by anatomic location. Perianal EMPD has a higher association with internal cancers, requiring thorough screening.
Area of Science:
- Oncology
- Dermatology
- Gastroenterology
Background:
- Extramammary Paget's disease (EMPD) can indicate underlying internal adenocarcinoma (secondary EMPD).
- The association between EMPD subtypes and adenocarcinoma, along with screening implications, requires further clarification.
Purpose of the Study:
- To determine the rates and types of adenocarcinomas associated with EMPD subtypes.
- To propose an evidence-based screening algorithm for detecting underlying adenocarcinomas.
Main Methods:
- A systematic literature review of 122 studies on EMPD published between January 1990 and November 2022.
- Multidisciplinary expert panel review of adenocarcinoma screening recommendations.
Main Results:
- Perianal EMPD showed a 25% association with adenocarcinoma (primarily colorectal).
- Penoscrotal and vulvar EMPD had a 6% association with adenocarcinoma (primarily genitourinary).
- Screening protocols vary by EMPD location, including colonoscopy, urine cytology, CT scans, mammography, and PSA tests.
Conclusions:
- Screening strategies for adenocarcinoma in EMPD patients must be tailored to the anatomic location of the EMPD.
- Anatomic subtyping of EMPD is crucial for guiding appropriate and effective screening protocols.
