COX-2 and Her-2/neu are overexpressed in Paget's disease of the vulva and the breast: results of a preliminary study

Arch Gynecol Obstet. 2008 Feb;277(2):135-8. doi: 10.1007/s00404-007-0434-1. Epub 2007 Aug 15.


Purpose: Paget's disease (PD) of the breast as well as the vulva is a rare condition that accounts for about 4% of breast neoplasms and 1% of vulvar malignancies. Recurrent disease after breast and vulvar surgery might be a challenge. To evaluate relevant molecules therapeutically, tissue from mammary and vulvar PD lesions was investigated immunohistochemically.

Methods: Histopathologic samples from 11 patients with mammary PD and eight patients with vulvar PD were stained with antibodies against estrogen and progesterone receptors, HER-2/neu and COX-2 followed by semiquantitative evaluation of the staining results.

Results: All tested mammary lesions as well as seven out of eight vulvar PD were negative for estrogen and progesterone receptors. Strong membranous staining for HER-2/neu (Score 3) was seen in all cases. Six out of 8 vulvar and 10 out of 11 mammary PD showed COX-2 overexpression.

Conclusions: PD of the breast and vulva are negative for estrogens and progesterons, therefore, anti-hormonal therapy is not indicated. The high frequency of Her-2/neu and COX-2 overexpression, however, suggests that these molecules could be therapeutically relevant in patients with PD. These results require further investigation.

MeSH terms

  • Breast Neoplasms / metabolism*
  • Cyclooxygenase 2 / metabolism*
  • Female
  • Humans
  • Immunohistochemistry
  • Paget Disease, Extramammary / metabolism*
  • Paget's Disease, Mammary / metabolism*
  • Receptor, ErbB-2 / metabolism*
  • Receptors, Estrogen / metabolism
  • Receptors, Progesterone / metabolism
  • Vulvar Neoplasms / metabolism*


  • Receptors, Estrogen
  • Receptors, Progesterone
  • Cyclooxygenase 2
  • PTGS2 protein, human
  • ERBB2 protein, human
  • Receptor, ErbB-2