Patients had a 2.67 times higher chance of local recurrence after WLE than MMS (95% confidence interval [CI]: 1.47, 4.85; p 5 .001).
Clinical Pearls
Studies investigating adults with diagnoses of Extramammary Paget’s Disease (EMPD) who underwent surgical intervention with follow up data were pooled and analyzed.
Patients had a 2.67 times higher chance of local recurrence after WLE than MMS (95% confidence interval [CI]:1.47, 4.85; p 5 .001).
Meta-analysis of single-arm studies revealed a 7.3% local recurrence rate after MMS (95% CI: 0.039, 0.107; p < .001) versus a 26.3% recurrence rate after WLE (95% CI:0.149, 0.376; p < .001).
Discussion:
Extramammary Paget Disease (EMPD) is an apocrine adenocarcinoma that is a rare, slow growing neoplasm. It is primarily found in the anogenital regions of elderly individuals. Of note, it can spread epidermotropically for years without significant invasion or metastasis. Treatment options include nonsurgical methods (including immunomodulators, topical chemotherapy, radiation) and surgery (wide local excision (WLE) and/or Mohs micrographic surgery (MOHS)). Historically, WLE has been presented as the gold standard for surgical management, however recurrence rates with this surgical treatment have been found to be particularly high. In addition, there is currently no established standard regarding the appropriate surgical margins in cases of EMPD surgical control. This study aimed to determine the rates of local recurrence for EMPD in adult patients undergoing MMS versus WLE through a systematic review and meta-analysis.
A literature search was performed with search terms including “extramammary Paget’s disease,” “Paget’s disease,” and “Mohs micrographic surgery.” All studies that met these search criteria were included. Further inclusion criteria consisted of age over 18 years, and those with a diagnosis of EMPD who underwent surgical intervention with follow up data. Exclusion criteria consisted of national database studies, non-English articles, systematic reviews, and single subject case reports.
In the single arm studies, EMPD has a 7.3% recurrence rate for all tumors when treated with MMS (95% confidence interval [CI]: 0.039, 0.107; p < .001) versus 26.3% when treated with WLE (95% CI: 0.149, 0.376; p < .001). When analyzing primary and recurrent tumor data separately in single arm studies, similar findings of improved outcomes were found, but not statistically significant.
When analyzing the double arm studies, the odds of recurrence was 2.67 (95% CI: 1.47, 4.85; p=.001) times higher after treatment with WLE compared with MMS. Furthermore, this was a significant finding with a nonsignificant heterogeneity (I2 = 0%, p .922).
This study provides novel and impactful findings regarding the use of WLE vs. MMS for surgical control of EMPD. Limitations included restriction of search to local recurrence only, lack of available prospective randomized control trials, exclusion of non-English articles into search criteria. Further research in the use of MMS in EMPD, specifically investigating different tumor stages and primary/recurrent status will be of great benefit to clinicians throughout.
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