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Extramammary Paget Disease.

J Marcoval1, R M Penín2, A Vidal2

  • 1Servicio de Dermatología, Hospital Universitari de Bellvitge, Barcelona, España.

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|April 13, 2020
PubMed
Summary
This summary is machine-generated.

Extramammary Paget disease (EMPD) is uncommon in Mediterranean populations. This study found dermal invasion and local recurrence are frequent, highlighting the need for long-term patient follow-up.

Keywords:
Enfermedad de Paget extramamariaExtramammary Paget diseaseIncidenceIncidenciaRecidivaRecurrenceTratamientoTreatment

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Extramammary Paget disease (EMPD) is a rare condition with limited data in Mediterranean populations.
  • This study reviews the clinical characteristics and outcomes of EMPD patients.

Purpose of the Study:

  • To characterize EMPD in a Mediterranean cohort.
  • To investigate the association with continuous neoplasms.
  • To analyze the long-term disease course and outcomes.

Main Methods:

  • Retrospective observational study of 27 EMPD patients diagnosed between 1990 and 2015.
  • Analysis of clinical and pathological findings, treatment, and outcomes.

Main Results:

  • The majority of patients were women, with lesions predominantly on the vulva.
  • Dermal invasion occurred in 41.7% of primary EMPD cases.
  • Local recurrence was observed in 29.6% of patients, and 11.1% died from metastasis.

Conclusions:

  • Underlying cutaneous adnexal adenocarcinoma is uncommon, but extracutaneous adenocarcinoma in continuity is possible.
  • EMPD frequently involves dermal invasion and can metastasize.
  • Long-term follow-up is crucial due to the common occurrence of delayed local recurrence.