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Extramammary Paget's disease. A critical reexamination
The American Journal of Dermatopathology
|January 1, 1979
Summary
Extramammary Paget's disease is not a single entity but likely originates as epidermal adenocarcinoma. It can spread to adnexal structures, dermis, or contiguous organs, with metastatic potential.
Area of Science:
- Dermatopathology
- Oncology
Background:
- Extramammary Paget's disease (EMPD) is a rare intraepidermal malignancy.
- Understanding its histological origins and spread is crucial for patient management.
Purpose of the Study:
- To classify extramammary Paget's disease based on histological findings.
- To elucidate the origins and patterns of spread of EMPD.
Main Methods:
- Histological examination of step-sections from 55 extramammary Paget's disease specimens.
- Clinical data and follow-up information were collected for 45 patients.
Main Results:
- Three distinct histological patterns of Paget cell distribution were identified: epidermal/adnexal, epidermal/adnexal/dermal, and involvement of contiguous organs.
- The majority of cases appeared to originate in the epidermis as an adenocarcinoma.
Conclusions:
- Extramammary Paget's disease represents diverse entities, often starting as epidermal adenocarcinoma.
- Spread can occur to adnexal structures, dermis (with metastatic potential), or contiguous organs like the genitourinary and gastrointestinal tracts.