Extramammary paget disease of the vulva with underlying mammary-like lobular carcinoma

A case report and review of the literature

Gabriel Villada, Uzma Farooq, Wendong Yu, John Paul Diaz, Clara Milikowski

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Extramammary Paget disease of the vulva accounts for 1%-2% of the neoplasms of the anogenital area. Very rarely, extramammary Paget disease of the vulva has been associated with an underlying mammary-like carcinoma, usually ductal, extremely rarely mixed ductal and lobular. We report the case of a 60-year-old female with a recurrent extramammary Paget disease of the vulva. Pathological examination of the wide excision of the vulva revealed an extramammary Paget disease with an underlying invasive carcinoma composed of medium size cells organized in single files, a morphology similar to that of an invasive lobular breast carcinoma. Immunohistochemical staining showed a comparable profile in the Paget cells and in the invasive tumoral cells: CEA and CK7 positivity; GCDFP-15, ER focal positivity. E-cadherin and HER2 were diffusely positive. S100 and CK20 were negative. HER2-CISH was amplified. The diagnosis of extramammary Paget disease of the vulva with an underlying mammary-like lobular carcinoma was made. Despite the characteristic lobular features, the immunohistochemical profile differs from the typical profile of a lobular carcinoma of the breast. The implications in term of prognostic and therapeutic significance need to be further studied.

Original languageEnglish
JournalAmerican Journal of Dermatopathology
DOIs
StateAccepted/In press - Apr 17 2014

Fingerprint

Extramammary Paget's Disease
Lobular Carcinoma
Vulva
Breast
Breast Neoplasms
Cadherins
Cell Size
Staining and Labeling
Carcinoma
Neoplasms

ASJC Scopus subject areas

  • Dermatology
  • Pathology and Forensic Medicine

Cite this

Extramammary paget disease of the vulva with underlying mammary-like lobular carcinoma : A case report and review of the literature. / Villada, Gabriel; Farooq, Uzma; Yu, Wendong; Diaz, John Paul; Milikowski, Clara.

In: American Journal of Dermatopathology, 17.04.2014.

Research output: Contribution to journalArticle

@article{742ee23226bb448e9648e03edf6e849a,
title = "Extramammary paget disease of the vulva with underlying mammary-like lobular carcinoma: A case report and review of the literature",
abstract = "Extramammary Paget disease of the vulva accounts for 1{\%}-2{\%} of the neoplasms of the anogenital area. Very rarely, extramammary Paget disease of the vulva has been associated with an underlying mammary-like carcinoma, usually ductal, extremely rarely mixed ductal and lobular. We report the case of a 60-year-old female with a recurrent extramammary Paget disease of the vulva. Pathological examination of the wide excision of the vulva revealed an extramammary Paget disease with an underlying invasive carcinoma composed of medium size cells organized in single files, a morphology similar to that of an invasive lobular breast carcinoma. Immunohistochemical staining showed a comparable profile in the Paget cells and in the invasive tumoral cells: CEA and CK7 positivity; GCDFP-15, ER focal positivity. E-cadherin and HER2 were diffusely positive. S100 and CK20 were negative. HER2-CISH was amplified. The diagnosis of extramammary Paget disease of the vulva with an underlying mammary-like lobular carcinoma was made. Despite the characteristic lobular features, the immunohistochemical profile differs from the typical profile of a lobular carcinoma of the breast. The implications in term of prognostic and therapeutic significance need to be further studied.",
author = "Gabriel Villada and Uzma Farooq and Wendong Yu and Diaz, {John Paul} and Clara Milikowski",
year = "2014",
month = "4",
day = "17",
doi = "10.1097/DAD.0000000000000065",
language = "English",
journal = "American Journal of Dermatopathology",
issn = "0193-1091",
publisher = "Lippincott Williams and Wilkins",

}

TY - JOUR

T1 - Extramammary paget disease of the vulva with underlying mammary-like lobular carcinoma

T2 - A case report and review of the literature

AU - Villada, Gabriel

AU - Farooq, Uzma

AU - Yu, Wendong

AU - Diaz, John Paul

AU - Milikowski, Clara

PY - 2014/4/17

Y1 - 2014/4/17

N2 - Extramammary Paget disease of the vulva accounts for 1%-2% of the neoplasms of the anogenital area. Very rarely, extramammary Paget disease of the vulva has been associated with an underlying mammary-like carcinoma, usually ductal, extremely rarely mixed ductal and lobular. We report the case of a 60-year-old female with a recurrent extramammary Paget disease of the vulva. Pathological examination of the wide excision of the vulva revealed an extramammary Paget disease with an underlying invasive carcinoma composed of medium size cells organized in single files, a morphology similar to that of an invasive lobular breast carcinoma. Immunohistochemical staining showed a comparable profile in the Paget cells and in the invasive tumoral cells: CEA and CK7 positivity; GCDFP-15, ER focal positivity. E-cadherin and HER2 were diffusely positive. S100 and CK20 were negative. HER2-CISH was amplified. The diagnosis of extramammary Paget disease of the vulva with an underlying mammary-like lobular carcinoma was made. Despite the characteristic lobular features, the immunohistochemical profile differs from the typical profile of a lobular carcinoma of the breast. The implications in term of prognostic and therapeutic significance need to be further studied.

AB - Extramammary Paget disease of the vulva accounts for 1%-2% of the neoplasms of the anogenital area. Very rarely, extramammary Paget disease of the vulva has been associated with an underlying mammary-like carcinoma, usually ductal, extremely rarely mixed ductal and lobular. We report the case of a 60-year-old female with a recurrent extramammary Paget disease of the vulva. Pathological examination of the wide excision of the vulva revealed an extramammary Paget disease with an underlying invasive carcinoma composed of medium size cells organized in single files, a morphology similar to that of an invasive lobular breast carcinoma. Immunohistochemical staining showed a comparable profile in the Paget cells and in the invasive tumoral cells: CEA and CK7 positivity; GCDFP-15, ER focal positivity. E-cadherin and HER2 were diffusely positive. S100 and CK20 were negative. HER2-CISH was amplified. The diagnosis of extramammary Paget disease of the vulva with an underlying mammary-like lobular carcinoma was made. Despite the characteristic lobular features, the immunohistochemical profile differs from the typical profile of a lobular carcinoma of the breast. The implications in term of prognostic and therapeutic significance need to be further studied.

UR - http://www.scopus.com/inward/record.url?scp=84899041444&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84899041444&partnerID=8YFLogxK

U2 - 10.1097/DAD.0000000000000065

DO - 10.1097/DAD.0000000000000065

M3 - Article

JO - American Journal of Dermatopathology

JF - American Journal of Dermatopathology

SN - 0193-1091

ER -