Primary extramammary Paget's disease: a clinicopathological study of 49 cases
YANG Xu-dan
FU Jing
WANG Lei
WANG Xiao-qing
LIU Yi-sha
ZHU Hong
SONG Lin-hong
XU Gang
Abstract：Objective To study the clinicopathological characteristics of extramammary Paget's disease (EMPD).Methods The routine HE,immunohistochemical staining and clinicopathological features were analyzed in 49 cases of EMPD with review of the literature.Results 36 cases were male and 13 patients were female.Patients aged 47 to 93 years,with average 68.3 years.Clinical findings were eczematoid lesions or neoplasm with significant pruritus and pain,skin lesions commonly involving the genitals,vulvar or anus,etc.Gross pathologic examination of EMPD revealed eczema,erythema or confluent papules with exudates and erosion.Histologically,EMPD was characterized by the presence of large oval or polyhedral intraepithelial Paget cells that had pale cytoplasm and large nuclei with prominent nucleoli.Paget cells were arranged either singly or in clusters throughout the epithelium,and sometimes formed gland-like structures.Immunohistochemically,Paget cells expressed CK7 and p16 in partial cases,p63 and CK5/6 were negitive.Follow-up showed 8 recurrence in 32 cases.Conclusions EMPD is a rare tumor with a male preponderance especially in perineum which has special histological features.Immunohistochemical stain can help for differential diagnosis from a variety of other skin carcinoma in situ.
Keywords：Extramammary Paget's diseaseClinicopathologyDifferential diagnosisImmunohistochemistry
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 245-248 )