A Comparative Study Of Clinicopathology In Mammary Paget’s Disease And Extramammary Paget’s Disease

Objetive : To investigate the clinical manifestations,histopathological features,immunophenotypes and the HER-2 gene status of the patients with mammary paget’s disease(MPD)and extramammary paget’s disease(EMPD)in the various indicators of the difference,with a view to raising awareness of them,and find reliable targeted therapy.Methods:By retrospective means,18 cases of Paget’s disease of breast and 17 cases of extramammary Paget’s disease were collected in pathology department of Huangshan People’s Hospital.Their clinicopathological features were sorted out,including age,course of disease,diameter of mass,lymphatic vessel invasion,lymph node metastasis/distant metastasis.Using routine HE staining,tissue microarray,immunohistochemical staining(IHC)and fluorescence in situ hybridization(FISH)techniques,the histomorphological changes,immunophenotype and gene status of human epidermal growth factor receptor 2(HER2)were observed.Results:1.Clinical data:(1)mammary Paget’s disease: a total of 18 cases,all women,the average age of patients 55.8 years(39~70 years old),11 cases of postmenopausal onset,the average diagnosis time required for 16.6 months,of which 13 cases involving the nipple,5 cases involving the areola The skin,both unilateral involvement,10 cases located on the right side and 8 cases on the left side.12 cases showed eczema-like changes,of which 13 cases of papillary hemorrhagic or hemorrhagic fluid disease,5 cases with itching or pain,17 cases palpable breast lumps,palpable axillary lymph nodes in 16 cases,17 cases of intraductal carcinoma or infiltration Cancer.Among them,There were 15 cases ofmodified radical mastectomy,2 cases with simple mastectomy and another underwent nipple biopsy.(2)Extramammary Paget’s Disease: A total of 17 cases,13 male patients and 4female,the ratio of male to female is about 3: 1.The average age of patients 62.3 years old(34 ~79 years old).The time required for diagnosis average 42.4 months.11 cases of scrotal involvement,1 case of perianal involvement,3 cases of genital involvement,1 case of labia,and 1 case of inguinal involvement.All showed eczema-like change.14 cases with itching or pain.1 case with inguinal lymph nodes.Surgery are selected local excision.(3)statistical comparison: There were significant differences in sex,concomitant tumors,recurrence and lymph node metastasis between Paget’s disease and extramammary Paget’s disease(P < 0.05).2.Histopathology:Paget cells are almost located in the epidermis,no obvious skin appendage involvement.Of the 18 cases with Paget’s disease,13 cases were accompanied by invasive breast cancer,4 cases were accompanied with ductal carcinoma in situ,2 cases were accompanied with ductal carcinoma in situ and early infiltration in the duct,and the other 1 case was Paget’s disease.4 cases of intravascular thrombus were found.There are 10 cases of lymph node metastasis.One case of 17 cases of extramammary Paget’s disease with lymph node enlargement,but no metastasis.One case of lesions adjacent to the side of the skin cut edge.3.Immunohistochemistry:(1)Paget cells in 18 cases of breast and Paget cells in 17 cases of Extramammary Paget’s disease were all expressed CK7,and were strongly positive;some cases expressed no specific difference between EMA,CEA and GCDFP-15;Cases of patients did not express CK20,CK5 / 6,p63,S-100 protein,HMB45,Melan-A;other special staining AB / PAS positive cases in 31 cases,less than 4 cases of unsatisfactory staining.(2)ER,PR in breast Paget disease were both low and not expressed,and only 2 patients had weak positive expression of ER in 1%;Ki-67 index ranged from 20% to 80%,and the median expression rate was about 60%.(3)The 18 cases of breast Paget’s disease in HER-2 protein positive expression(13/18),only 5 cases were not expressed.The group of 17 cases of Extramammary Paget disease also see HER-2 varying degrees of positive(12/17).4.Fluorescence in situ hybridization(FISH):In 18 cases of breast Paget’s disease,HER-2 gene amplification rate was 66.6%(12/18),6 cases without amplification.The positive coincidence rate of HER2 gene amplification in cases of HER2 protein / C-erbB-2 expression 2+ and 3+ was coincide.In addition,in cases of Paget’s disease with intraductal carcinoma and invasive carcinoma,the expression of Her-2 in the intraductal carcinoma cells is basically consistent with the expression of HER-2 in Paget cells.17 cases of Extramammary Paget disease HER-2 gene amplification rate of 52.9%(9/17),significantly lower than the Paget disease of the breast.Conclusion:(1)There are some similarities in clinical and pathological features between Paget’s disease and extramammary Paget’s disease.(2)Human epidermal growth factor receptor-2(HER-2)is highly expressed in Paget’s disease and extramammary Paget’s disease.(3)HER-2 can be used as an immunohistochemical marker for the diagnosis and differential diagnosis of extramammary Paget disease.