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Perianal Pagetoid Intraepithelial Carcinoma
Suanga Sisodia1, Robin Boushey2, Goo Lee3
1aDivision of Radiation Oncology, The Ottawa Hospital and the University of Ottawa, Ottawa, Ontario, Canada.
Extramammary Paget disease (EMPD) of the perianal region is rare. This case highlights aggressive surgical management for perianal pagetoid intraepithelial carcinoma, emphasizing the need for improved risk stratification in managing this challenging condition.
Area of Science:
- Oncology
- Dermatology
- Gastroenterology
Background:
- Extramammary Paget disease (EMPD) in the perianal region is uncommon and difficult to treat.
- Primary EMPD presents as noninvasive epidermal lesions, while secondary EMPD arises from underlying malignancies.
- Distinguishing between primary and secondary EMPD can be challenging without detected malignancies.
Observation:
- A 74-year-old woman presented with perianal pagetoid intraepithelial carcinoma.
- Initial workup did not reveal an obvious underlying malignancy.
- The patient opted for aggressive surgical management.
Findings:
- The case underscores the rarity and complexity of managing perianal EMPD.
- Aggressive surgical intervention was chosen despite the absence of a detected primary tumor.
- Continued case reporting is crucial for improving risk stratification and treatment strategies.
Implications:
- Further research and case studies are needed to refine diagnostic criteria for perianal EMPD.
- Optimal management strategies for perianal EMPD, especially with suspected occult cancers, require further elucidation.
- Early and aggressive management may be warranted in select cases of perianal EMPD to improve patient outcomes.
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