Vulvar Paget disease

The EM forms of Paget disease occur predominantly in females as vulvar or perianal disease but may also occur in males and at other sites. 

Primary vulvar Paget disease is a localized carcinoma of sweat duct origin that may be in situ or invasive histologically, it is composed of large cells with voluminous cytoplasm containing mucin that are universally CK7+ and GCDFP-15+.329... 

Vulvar Paget disease, primary adnexal carcinoma CK7+, CCDFP-15+, CEA+... 

Metastatic colorectal carcinoma presenting as perianal-vulvar Paget disease CK7 (negative), CK20+, CDX2+, CEA+, CCDFP-15 (negative)... 

FIGURE 18.1 Primary vulvar Paget disease (A). (H E.) Tumor cells express CK7 (B) and GCDFP (C), but normal epithelium does not. Occult stromal invasion can be highlighted by CK7 (D) when inflammation obscures the epidermal-dermal junction. 

Primary vulvar Paget disease is typically positive for CK7 and CCDFP, and negative for CK20 and CDX2. 

Secondary vulvar Paget disease of colorectal or urothelial origin shows similar immunophenotype as the tumor at the primary site. 

Brown HM, Wilkinson EJ. Uroplakin-III to distinguish primary vulvar Paget disease from Paget disease secondary to urothelial carcinoma. Hum Pathol. 2002 33 545-548. 

Goldblum JR, Hart WR. Vulvar Paget s disease A clinicopatho-logic and immunohistochemical smdy of 19 cases. Am J Surg Pathol. 1997 21 1178-1187. 

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