Clinical Features And Prognosis Of Extramammary Paget’s Disease

Background and purposeExtramammary Paget’s disease(EMPD),also known as eczema-like carcinoma of the breast,is a rare malignant skin tumor.EMPD tends to occur in scrotum,vulva,penis,perineum and perianal areas,mainly manifested as pruritus,erythema,pain,erosion,exudation and dander.EMPD is easily misdiagnosed as eczema,dermatitis and other skin diseases in the early stage,thus delaying treatment.Mohs microsurgery has certain benefits for patients,such as lower recurrence rates and longer disease-free survival(DFS).This study was designed to investigate the clinical characteristics,risk factors for recurrence and prognostic factors of EMPD,which may provide a basis for the diagnosis and treatment of EMPD to some extent.MethodRetrospective statistical analyses were conducted on the medical records of 41 patients diagnosed with EMPD from 2009 to 2019.The survival status was followed up.The relationship between the age of onset,gender,course of disease,lesion area,surgical margin,with or withouttumor cell infiltration,pathological margin positive or negative,with or without lymph node metastasis,with or without underlying tumor and the prognosis was analyzed.ResultsAmong all the patients,34(82.93%)were male and 7(17.07%)were female,and the onset age was 50-85 years.Thirty-eight patients(92.68%)received surgical resection,17 invasive patients(44.74%)included.Recurrence occurred in 8 of the 38 patients,4 of whom died.Age,gender,course of disease and surgical margin had no significant effect on the survival of patients(p > 0.05),while large lesion area,positive surgical margin,tumor cell infiltration and lymph node metastasis all increased the DFS of patients(p < 0.05).ConclusionEMPD often occurs in elderly men and is characterized by a long course of disease,easy recurrence and invasion.EMPD is often misdiagnosed as eczema and dermatitis in the early stage due to the characters of skin lesions,.Surgical resection is the first choice for treatment.Large lesion area,positive surgical margins,lymph node metastasis,invasive growth and distant metastasis are important risk factors for recurrence and prognosis.