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Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.

Nour Kibbi1, Joshua L Owen2, Brandon Worley3

  • 1Department of Dermatology, Stanford University School of Medicine, Redwood City, California.

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|January 20, 2022
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Summary
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Extramammary Paget disease (EMPD) requires accurate diagnosis via skin biopsies and baseline screening for internal cancers. Treatment varies by stage, with surgery preferred for invasive EMPD, and close follow-up is crucial for recurrence monitoring.

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Area of Science:

  • Dermatology
  • Oncology
  • Clinical Practice Guidelines

Background:

  • Extramammary Paget disease (EMPD) is a recurrent malignant neoplasm with metastatic potential.
  • EMPD often affects older adults on genital, perianal, and axillary skin.
  • It can be associated with internal malignant neoplasms.

Purpose of the Study:

  • To develop evidence-based clinical care recommendations for adult EMPD patients.
  • To provide guidance on diagnosis, staging, and treatment of EMPD.

Main Methods:

  • Systematic literature review of EMPD from January 1990 to September 2019.
  • Analysis of 483 studies.
  • Multidisciplinary expert panel evaluation to formulate recommendations.

Main Results:

  • Diagnosis requires multiple skin biopsies; baseline screening for internal malignancies is essential.
  • Sentinel lymph node biopsy/dissection is not routinely recommended.
  • Surgical resection is preferred for invasive EMPD; various treatments exist for intraepidermal or unresectable cases.
  • Chemotherapy or targeted therapy for metastatic disease; close follow-up for recurrence is advised.

Conclusions:

  • Clinical practice guidelines offer direction on EMPD diagnosis and management.
  • Further research via prospective registries can enhance understanding of EMPD natural history and treatment efficacy.