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

Abstract

PurposeThere 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 MethodsFrom 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).ResultsThe 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.ConclusionsDiagnosis 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.

Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature

“…Interestingly, nine of the 20 patients had a second malignancy. A series of 25 Chinese and 19 South Korean penoscrotal EMPD patients showed only 1 and 2 deaths due to disease, respectively [ 10 , 12 ].…”

R Inguinal/R Scrotum Extramammary Paget’s Disease with Diffuse Spine Metastasis Complicated by Microangiopathic Hemolytic Anemia

“…In a cohort of the 19 patients with genital EMPD in South Korea, only three of the patients were noted to have an underlying malignancy (colorectal, cholangiocarcinoma, and parotid gland, resp.) [ 7 ]. In our patient, computed tomography of the chest, abdomen, and pelvis was negative for the underlying malignancy.…”

Extramammary Paget's disease: Surgical control from the plastic surgery perspective

“…15,16 Strategies that have been employed in tackling this problem of local recurrence after surgical excision have included Mohs surgery, 17,18 the use of intraoperative frozen section and taking recommended margins from the grossly-visible edges of the lesion. [19][20][21][22] All three approaches can theoretically take care of the clearance of the tumour per se, but not of the field change effect. For example, frozen section has not been unanimously reported to be beneficial.…”

“…Less often discussed but also salient is the effect of field change known with EMPD. The presence of multicentric foci, skip lesions15,16 and therefore the essentially non-contiguous nature of the disease means that disease may not have been totally eradicated despite pathologically clear margins.Strategies that have been employed in tackling this problem of local recurrence after surgical excision has included Mohs surgery[17][18] , use of intra-operative frozen section, and the recommendation of taking recommended margins from the grossly visible edges of the lesion[19][20][21][22] .…”