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Genitourinary extramammary Paget’s disease: review and outcome in a multidisciplinary setting

  • Phyo, Aung Kyaw1
  • Mun, Kein-Seong2
  • Kwan, Kong Chee3
  • Ann, Chai Chu1
  • Kuppusamy, Shanggar1
  • 1 Division of Urology, Department of Surgery, University of Malaya, Kuala Lumpur, Malaysia
  • 2 Department of Pathology, University Malaya, Kuala Lumpur, Malaysia
  • 3 Division of Plastic and Reconstructive Surgery, Department of Surgery, University of Malaya, Kuala Lumpur, Malaysia
Published Article
International journal of clinical and experimental pathology
Publication Date
Sep 01, 2020
PMID: 33042345
PMCID: PMC7539884
PubMed Central


Background: Extramammary Paget’s disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis. Objective: To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution. Methods: Retrospective observation data analysis of 19 EMPD cases was performed. Demographic information, clinical management records, and histopathologic data of individual cases were obtained from the inpatient hospital data registry. Results: The mean age (years) at time of diagnosis was 62.4 with equal gender distribution. Synchronous tumors were detected in 6 cases (31.5%). 18 out of 19 patients underwent definitive surgical management in the form of wide local excision (WLE) and reconstructive surgery. Positive margins were found in 11 (68.8%) cases and 7 out of these 11 cases underwent second look surgical intervention to achieve oncological clearance or adjuvant oncology treatment. Follow-up period for living patients varied depending on time of diagnosis and definitive treatment. 10 out 19 cases (52.7%) were alive at the time of the study. Among the 7 cases of mortality from cancer, 5 cases died from progression of underlying associated malignancy and only 2 cases died with advanced stage of EMPD. Conclusion: EMPD can be quite aggressive, especially in the secondary form, and surgical management is challenging with a high rate of residual tumor at the surgical margin. EMPD can easily mislead the clinician and patient, leading to unnecessary delay prior to definitive effective management.

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