Mucosal Invasion, but Not Incomplete Excision, Has Negative Impact on Long-Term Survival in Patients With Extramammary Paget’s Disease

Abstract

BackgroundExtramammary Paget’s disease (EMPD) sometimes spreads from the skin to mucosal areas, and curative surgical excision of these areas is challenging. The aim of this study is to analyze the impact of mucosal involvement and surgical treatment on the survival of patients with EMPD.MethodsWe conducted a retrospective review of 217 patients with EMPD. We also assessed the associations between tumor involvement in boundary areas (anal canal, external urethral meatus, vaginal introitus), prognostic factors, and survival in 198 patients treated with curative surgery.ResultsOf 217 patients, 75 (34.6%) had mucosal boundary area involvement. Lesions in these areas were associated with frequent lymphovascular invasion (p = 0.042), lymph node metastasis (p = 0.0002), incomplete excision (p < 0.0001), and locoregional recurrence (p < 0.0001). Boundary area involvement was an independent prognostic factor associated with disease-specific survival, per multivariate analysis (HR: 11.87, p = 0.027). Incomplete excision was not significantly correlated with disease-specific survival (HR: 1.05, p = 0.96).ConclusionBoundary area tumor involvement was a major risk factor for incomplete excision, local recurrence, and poor survival outcomes. However, incomplete removal of primary tumors was not significantly associated with poor prognosis. A less invasive surgical approach for preserving anogenital and urinary functions may be acceptable as the first-line treatment for resectable EMPD.

Prognostic Analysis of Patients With Extramammary Paget Disease Treated With Conservative Excision

“…The prognosis of EMPD is relatively good, 6 and it has been reported that incomplete excision of primary tumors does not statistically correlate with worse disease-specific survival nor is such excision a poor prognostic factor, compared with complete removal. 12 In this study, there were no significant differences in OS or MFS between the conservative excision group and the wide excision group, which suggests that local management of the primary tumor is not directly related to EMPD metastasis and prognosis. In addition, the subgroup analysis of the cases with a follow-up period of at least 24 months revealed that there were no significant differences in OS between the 2 groups, although MFS was shorter for the conservative excision group.…”

Functional preservation benefits of minimal surgery for extramammary Paget's disease

“…There are few reports on surgical margins and survivalof EMPD. Some studies reported no association between surgical margins and survival in patients with vulvar EMPD 14,15 …”

Trop2 Expression in Extramammary Paget’s Disease and Normal Skin

“…In clinical settings, EMPD is curable when surgically eradicated in the early stage because the tumors generally exhibit slow growth and a long dormant phase [ 4 , 44 ]. However, complete surgical removal is sometimes difficult because of diagnostic delays or anatomical constraints, leading to an increased risk of metastasis [ 4 , 10 ]. Conventional chemotherapy with taxanes, platinum-containing drugs, 5-fluorouracil, epirubicin, vincristine, and mitomycin C has been to treat metastatic EMPD, but the efficacy is unsatisfactory [ 4 , 11 ].…”

“…Approximately 15–40% of EMPD lesions exhibit dermal invasion in their clinical course, increasing the risk of lymph node and distant metastasis [ 2 , 4 ]. Although complete surgical removal is the treatment of choice for resectable EMPD, this is sometimes difficult to achieve because of the inconspicuous tumor border and anatomical constraints [ 9 , 10 ]. The prognosis of unresectable EMPD is poor once the tumor metastasizes because of the lack of effective systemic therapies [ 4 , 11 , 12 , 13 , 14 ].…”