Apocrine adenocarcinoma is a rare malignancy with invasive potential. Its clinical behavior and histologic appearance are highly variable, making accurate diagnosis difficult. We report on a case of apocrine carcinoma presenting with lymph node infiltration and extensive extramammary Paget's disease. Wide local excision with lymph node dissection is the mainstay of treatment. The role of adjuvant therapy has yet to be established.
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