Extramammary Paget’s disease (EMPD) is a rare malignancy, and little was known about its prognostic factors and optimal treatment. In the current study, we aimed to discuss clinical and pathological features of scrotal EMPD and determine the prognostic factors for cancer-speci c survival and local recurrence. A total of 206 patients with scrotal EMPD lesions surgically treated at our institute were studied. All clinical and pathological data were reviewed. Immunohistochemical staining of TP53 and Ki67 was examined as well. At the last follow-up, 175 patients (84.95%) were alive. Twelve patients (5.83%) had died of the disease due to distant metastases. Fifteen patients (7.28%) developed
local recurrences of scrotal EMPD. Ki67 expression was signi cantly elevated in patients with wide horizontal invasion (P = 0.003). In univariate analysis, high invasion level, presence of nodule, presence of lymphovascular invasion, adnexa invasion, lymph node metastasis and high p53 expression were signi cant factors for poor cancer-speci c survival. In multivariate analysis, high p53 expression was signi cantly correlated with poor cancer-speci c survival. Wide horizontal invasion was independently correlated with local recurrence-free survival of scrotal EMPD. In conclusion, wide horizontal invasion is an independent risk factor for local recurrence-free survival in the patients with scrotal EMPD.
Extramammary Paget’s disease (EMPD) is a rare malignancy that mainly a ects the anogenital region in elderly people. In men, the scrotum is more involved than the penis, and the disease is usually misdiagnosed as eczema. It is considered to be most likely derived from the undifferentiated pluripotent cells of the epidermis1. e disease can be classified as primary or secondary EMPD. Primary EMPD arises as an in situ tumor in the epidermis, while secondary EMPD involves direct expansion to the skin from underlying neoplasm, commonly a rectal or genitourinary carcinoma2.
Most patients with primary EMPD have a good prognosis, because the tumor cells grow slowly and the lesion is usually limited to the epidermis3. However, in some cases the tumor can present aggressive behavior and invades the dermis and subcutaneous tissue. Once the tumor invades the dermis, the risk of lymph node metas- tasis increases, and could result in a poor prognosis4,5. With regard to treatment, complete surgical excision is the rst choice for patients with primary EMPD without distant metastasis and a complete cure can be expected in most cases6,7. On the contrary, the treatment e ect for invasive EMPD with metastasis is o en disappointed as no standardized highly e ective therapy has been developed presently8.
e method of surgical excision and de ning the surgical margin of EMPD remain controversial. At present, surgical modalities including Mohs micrographic surgery, uorescent dyes and frozen section examination (FSE) are recommended to ensure clear margins. However, even extensive resections are complicated by a high local recurrence rate due to several characteristics including tumor multifocality and ill-de ned margins9,10.
Material and Methods
A total of 206 patients with scrotal EMPD lesions were included in this study. All patients were surgically treated between April 2003 and May 2015 at the department of Urology of Huashan Hospital. All lesions were primary EMPD and the cases of secondary EMPD were excluded.