乳腺外Paget’s病

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乳腺外Paget’s病

Whenever you make a diagnosis of vulva or anal Paget's disease, you have not done your job yet! Your next step is to make sure this is a primary, not a secondary vulva/anal Paget's disease. Since secondary vulva/anal Paget's is often associated with underlying internal malignancy from GI tract, urothelia or other sources, whenever you make a diagnosis of vulva/anal Paget's disease, you are obligated to exclude these malignancies. The prognosis of primary vulva Paget's is far more favorable than secondary vulva Paget's. If patient has co-existing vulva and anal Paget's disease or if vulva Paget's disease has perineal or perianal spreading, the chance associated with underlying internal malignancy is increased. Therefore, the number one task for diagnosing a lesion like this case is to rule out secondary vulva/anal Paget's disease (although only counts for about 5% of all vulva Paget's disease!)! You may want to ask clinician if there is perianal involvement of this lesion. There are some immunohistochemical markers which can help us differentiate, such as CK7, CK20, GCDFP, and S100. Did you do immunostainings to rule out secondary vulva Paget's disease?

 补充：

1 约10~20%外阴Paget病伴有浸润癌或皮肤附属器腺癌（WHO 2003）

2 除上述CK7、CK20、GCDFP和S100外，CEA、p63、p16、uroplakin III等标记物以及PAS或粘液卡红等特殊染色，均有助于鉴别。