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[Extramammary Paget's disease].

I Cosgarea1, A Zaremba2, U Hillen3

  • 1Dermatological Sciences, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, Großbritannien.

Der Hautarzt; Zeitschrift Fur Dermatologie, Venerologie, Und Verwandte Gebiete
|September 5, 2019
PubMed
Summary
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Extramammary Paget's disease (EPD) is a rare skin cancer that can mimic inflammatory conditions, delaying diagnosis. Surgical excision is the primary treatment for localized EPD, while advanced stages have a poor prognosis.

Area of Science:

  • Dermatology
  • Oncology

Background:

  • Extramammary Paget's disease (EPD) is a rare cutaneous adenocarcinoma.
  • It is characterized by epidermal Paget's cells, similar to mammary Paget's disease.
  • EPD is classified into primary (Type I) and secondary (Type II and III) forms associated with other carcinomas.

Purpose of the Study:

  • To review the clinical presentation, diagnosis, and treatment of Extramammary Paget's disease.
  • To highlight diagnostic challenges and therapeutic options for EPD.

Main Methods:

  • Review of existing literature on Extramammary Paget's disease.
  • Analysis of histological characteristics and clinical classifications.
  • Discussion of treatment modalities for localized and metastatic EPD.
Keywords:
Adnexal infiltrationCutaneous adenocarcinomaExcisionMicroscopically controlled surgeryPaget’s carcinoma

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Main Results:

  • EPD presents unspecifically, often mimicking chronic inflammatory skin disorders, which can delay diagnosis.
  • Micrographically controlled excision is the treatment of choice for localized EPD.
  • Histological assessment of adnexal infiltration is crucial before topical therapies.
  • Complete tumor excision can be difficult due to ill-defined borders.
  • Metastatic EPD has a poor prognosis, and controlled clinical trials for systemic treatment are lacking.

Conclusions:

  • Early and accurate diagnosis of EPD is essential for effective management.
  • Surgical excision remains the cornerstone of treatment for localized disease.
  • Further research is needed for effective systemic treatments for metastatic EPD.