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

Extramammary Paget's disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis.

To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution.

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.

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.

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.