A Primer on Extramammary Paget's Disease for the Urologist

Joon Yau Leong; Paul H. Chung


Transl Androl Urol. 2020;9(1):93-105. 

In This Article

Course and Prognosis

Patients with primary or intraepithelial EMPD have a favorable prognosis, with studies showing that the mortality rates of patients treated adequately for non-invasive disease do not differ significantly from that of the general matched population.[30] Primary disease, though seemingly treatable, has the potential to disseminate and become invasive.[31] Overall mortality rates for the secondary form of EMPD have been reported to be 26–66%, either from metastatic EMPD or from their associated internal malignancies.[19] Moreover, mortality rates were found to be significantly higher in patients with an underlying adnexal carcinoma when compared to those who did not (46% vs. 18%, P<0.05).[12]

Several factors such as dermal invasion, elevated CEA levels and lymph node metastasis portend a greater risk of adverse prognosis, with the former being the most significant. Hatta found that the 5-year survival rate for patients with deep invasion beneath the reticular dermis was significantly lower than patients with no invasion or with microinvasion to the papillary dermis.[32] Histological assessment is therefore needed for appropriate risk stratification.[33–35] Serum CEA levels have also been associated with predicting systemic metastasis in EMPD patients with a sensitivity of 70% and specificity of 94%.[36] CEA may be used as an indicator to monitor treatment effects and outcomes for EMPD.[37,38] Regional lymph node metastasis has also been shown to significantly affect the prognosis of EMPD. Unfortunately, limited studies have evaluated the utility of lymphadenectomy as a treatment strategy for EMPD.[32,34] Further assessment of lymphadenectomy should be performed before it can be accepted as a standard treatment. Studies have also examined the efficacy of sentinel lymph node biopsy (SLNB) and reported a significant association between SLN positivity and increased dermal invasion rates, lower overall 5-year survival rates, and higher lymphovascular metastatic rates.[39–44] Due to the limited existing data regarding lymphadenectomy, chemotherapy, immunotherapy and radiation therapy in EMPD disease, patients with metastatic disease should be evaluated via a multidisciplinary approach to develop a treatment plan that may maximize outcomes for individual patients.