Extramammary Paget's disease: an annotated review.

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Extramammary Paget's disease (EMPD) is an uncommon neoplasm of the skin.
• It is frequently associated with underlying adnexal neoplasms or distant organ malignancies.
• EMPD possesses malignant potential, capable of metastasis even without a detectable primary cancer.

Purpose of the Study:

• To review the incidence, clinical features, histogenesis, and treatment of extramammary Paget's disease.
• To elucidate the cell of origin for EMPD.
• To assess current and potential treatment strategies for EMPD.

Main Methods:

• Review of existing literature on extramammary Paget's disease.
• Analysis of histochemical, immunohistological, and lectin binding studies.
• Evaluation of treatment outcomes, including surgical excision, chemosurgery, radiation therapy, and chemotherapy.

Main Results:

• EMPD is associated with underlying adnexal neoplasms in approximately 50% of cases.
• A higher than expected incidence of distant organ malignancies is observed in EMPD patients.
• Studies suggest EMPD originates from exocrine sweat gland cells, with apocrine derivation being more common than eccrine.
• Wide margin excision shows a high recurrence rate; chemosurgery may reduce local relapse.
• The efficacy of adjuvant radiation therapy is undetermined; chemotherapy has shown promise in advanced cases.

Conclusions:

• EMPD is a potentially malignant condition with diverse origins and associations.
• Effective management requires addressing both the primary lesion and potential underlying or metastatic disease.
• Further clinical trials are needed to establish optimal treatment protocols, particularly for chemotherapy.