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Extramammary Paget disease is an uncommon cutaneous malignancy that primarily affects areas rich in apocrine glands. Here, we aim to present an 84-year-old woman with a distinctive perianal neoplastic process comprised of conventional Paget disease with an intertwined in-situ glandular component. Rare foci of glands embedded in pools of mucin were also identified in the most recent excision, consistent with mucinous adenocarcinoma.

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Recommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic subtype.

J Am Acad Dermatol

October 2024

Department of Dermatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois. Electronic address:

Article Synopsis
  • Extramammary Paget's disease (EMPD) can be linked to underlying internal adenocarcinomas, with varying associations based on the EMPD subtype.
  • A systematic literature review identified that perianal EMPD has a higher rate (25%) of associated adenocarcinomas compared to penoscrotal and vulvar types (6% each).
  • The proposed screening algorithms suggest specific tests based on EMPD subtype, emphasizing thorough screening for high-risk perianal cases and more cost-effective approaches for lower-risk penoscrotal and vulvar cases.
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Hematoporphyrine injection-based photodynamic therapy for Perianal Paget's disease: A case report.

Photodiagnosis Photodyn Ther

December 2024

Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of Medicine, Shanghai, China. Electronic address:

Article Synopsis
  • Paget's disease of the perianal skin (PPD) is a rare skin condition that can be caused by issues in the colon or develop on its own.
  • Surgery is usually the main treatment for PPD, but it can sometimes cause problems and isn't always a good option for older patients or those with severe cases.
  • An alternative treatment called HpD-PDT helped a 70-year-old man with this condition by getting rid of the lesions without harming his ability to use the bathroom or causing other side effects.
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Article Synopsis
  • Paget disease of the breast is a type of cancer affecting the nipple skin, while extramammary Paget disease occurs in other skin areas like the pubis and perianal regions.
  • A case study is presented about a Japanese woman in her 70s who had two ectopic extramammary Paget disease lesions on her chest and buttock, which were surgically removed.
  • This case is notable as it marks only the third instance of multiple simultaneous ectopic extramammary Paget disease and highlights the diagnostic challenges due to its unusual locations.
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Article Synopsis
  • A panel-based immunohistochemistry approach was used to subtype perianal Paget disease (PPD) and investigate the effectiveness of SATB2 as a marker for secondary PPD.
  • In a study of 15 PPD cases, 53% were identified as secondary PPD, revealing distinct staining patterns, where SATB2 and CDX2 markers were key in differentiation.
  • The findings suggest that combining IHC panels with clinical and imaging data enhances classification accuracy for PPD, with SATB2 serving as a valuable tool alongside CDX2.
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