title: Clinical and pathological characteristics of extramammary Paget's disease: report of 246 Chinese male patients.
abstract: Extramammary Paget's disease (EMPD) is a rare cutaneous neoplasm. The aim of this study was to elaborate the clinical and pathological features of Chinese EMPD male patients. The study comprised 246 patients with EMPD at our institute from January 1993 to December 2012. Scrotum was the most common initial site. The average age of onset was 63.9 years but the mean delay in diagnosis was 3.6 years. EPMD spread exclusively to the inguinal lymph nodes and the right inguinal lymph nodes are more likely to suffered Paget cells infiltration. Accompanying malignancies were found in 20 patients. Pathological examination revealed 63 patients defined as invasive EMPD. Immunohistochemical detection showed various expression levels of EMA, CEA, CK7, HER2/neu, Ki67, P53, CK20 and S100 in tumor tissues, but negative expression of VIM, LCA and HMB45. HER2/neu protein exhibited a significant association with invasive EMPD. A novel histological type of EMPD with CK7-/S100+ was identified. Elevated serum PSA level was observed in only 16% patients. Invasive EMPD often had advanced age of onset. Metastatic EMPD showed significantly shorter in the delay in diagnosis and the greater length of skin lesion in contrast to others. This study demonstrates the clinical and pathological features of Chinese male EMPD patients, and may provide implications for the management of Chinese EMPD patients.
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Zhihua
authors.initials: Z
authors.lastname: Kang
authors.affiliation: Department of Dermatology, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Qiaoan
authors.initials: Q
authors.lastname: Zhang
authors.affiliation: Department of Laboratory Medicine, The Fifth People's Hospital of Shanghai, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Qunfeng
authors.initials: Q
authors.lastname: Zhang
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital North, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Xiangyu
authors.initials: X
authors.lastname: Li
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Tingting
authors.initials: T
authors.lastname: Hu
authors.affiliation: Central Laboratory, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Xiao
authors.initials: X
authors.lastname: Xu
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital North, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Zhiyuan
authors.initials: Z
authors.lastname: Wu
authors.affiliation: Central Laboratory, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Xinju
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Hua
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Dermatology, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Jinhua
authors.initials: J
authors.lastname: Xu
authors.affiliation: Department of Dermatology, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Feng
authors.initials: F
authors.lastname: Xu
authors.affiliation: Department of Laboratory Medicine, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China ; Department of Laboratory Medicine, Huashan Hospital North, Shanghai Medical School, Fudan University Shanghai, P. R. China ; Central Laboratory, Huashan Hospital, Shanghai Medical School, Fudan University Shanghai, P. R. China.
authors.forename: Ming
authors.initials: M
authors.lastname: Guan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism; D002681:China; D006801:Humans; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000378:metabolism / Q000473:pathology*; D011379:Prognosis; D017430:Prostate-Specific Antigen / Q000097:blood; D012611:Scrotum / Q000378:metabolism / Q000473:pathology*; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D017430:Prostate-Specific Antigen
keywords: Extramammary Paget’s disease; invasion; metastases; pathology