Korean J Urol.  2013 Dec;54(12):834-839.

Extramammary Paget Disease of External Genitalia: Surgical Excision and Follow-up Experiences With 19 Patients

• Jung JH
• Kwak C
• Kim HH
• Ku JH

Abstract

PURPOSE
There are only a few reports of extramammary Paget disease (EMPD) of the external genitalia because it is a rare malignancy. We investigated patients with EMPD of the penis and scrotum and report the outcome of surgical management.
MATERIALS AND METHODS
From 2000 to 2012, a total of 19 patients diagnosed as having penile and scrotal EMPD underwent wide local excision with or without intraoperative frozen biopsy or preoperative mapping biopsy. The medical charts of these patients were reviewed and analyzed retrospectively. Mean follow-up was 22.5 months (range, 1 to 60 months).
RESULTS
The mean age of the patients was 68 years (range, 57 to 82 years). In some patients, the lesions were misdiagnosed as either eczema or some other benign skin lesion at outside institutions, with a mean delay in diagnosis of 43.5 months (range, 1 to 198 months). Intraoperative frozen biopsy or preoperative mapping biopsy was performed in 18 patients. The resection margin was negative in 9 patients (47.4%) and positive in 10 patients (57.6%). Intraepithelial EMPD without dermis invasion was observed in 5 patients (26.3%), whereas diseases with dermis invasion were noted in 14 patients (73.7%). During the follow-up period, recurrences occurred in four patients, and two patients with dermis invasion and recurrence died from the disease.
CONCLUSIONS
Diagnosis of EMPD should not be delayed to allow for prompt management. Our findings suggest that intraoperative frozen biopsy or preoperative mapping biopsy cannot guarantee negative margins on final pathology. However, preoperative mapping biopsy and wide local excision with intraoperative frozen biopsy demonstrates good prognosis of EMPD, especially in those cases without dermal invasion.

Keyword

External genitalia; Extramammary Paget disease; Penis; Scrotum; Surgery

MeSH Terms

Biopsy
Dermis
Diagnosis
Eczema
Follow-Up Studies*
Genitalia*
Humans
Male
Paget Disease, Extramammary*
Pathology
Penis
Prognosis
Recurrence
Retrospective Studies
Scrotum
Skin

Figure

• 

  FIG. 1 Location of mapping biopsies marked 2 cm distal to the gross margin. (A) Erythematous lesion of extramammary Paget disease at the junction of the penis and scrotum. The margin of the lesion is identified grossly. The dashed mark represents the location of punch biopsies and the dots represent a 2-cm safety margin. (B) Numbers 22 and 23 represent the lesion of additional punch biopsy because number 19, which was the site of the initial punch biopsy, was extramammary Paget disease (+) on the pathologic report.

• 

  FIG. 2 Kaplan-Meier curve. Kaplan-Meier curve for recurrence-free survival (A) and overall survival (B).

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