For DFSP, MMS is associated with a lower rate of recurrence than WLE and is particularly useful for tumors on the head and neck. Imatinib is used in advanced disease and may take on a larger role in the treatment of DFSP in the future.
BACKGROUND
Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence.
OBJECTIVE
To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis.
MATERIALS AND METHODS
Literature review using PubMed search for articles related to EMPD.
RESULTS
Extramammary Paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy.
CONCLUSION
Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.
Surgery is the preferred treatment for local disease. Limited data suggest that Mohs micrographic surgery may provide superior clinical outcomes, but more research is needed regarding the long-term outcomes. Radiation and systemic chemotherapy are reserved for recurrent or metastatic disease.
BACKGROUND
Extramammary Paget's disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine-rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence.
OBJECTIVE
To review the current literature on EMPD regarding treatment and prognosis.
MATERIALS AND METHODS
Literature review using PubMed search for articles related to EMPD.
RESULTS
Extramammary Paget's disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy.
CONCLUSION
Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.
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