Mariam Hina, Asra Hasan, Maria Tariq, Muhammad Muaz Abbasi, Muneeb Uddin Karim
{"title":"Multi-Disciplinary Management of Hidradenocarcinoma","authors":"Mariam Hina, Asra Hasan, Maria Tariq, Muhammad Muaz Abbasi, Muneeb Uddin Karim","doi":"10.21089/njhs.83.0128","DOIUrl":null,"url":null,"abstract":"Hidradenocarcinoma makes up 6% of eccrine malignancies, and is an uncommon eccrine sweat gland tumour. The etiology is due to genetic alterations and transformation from an existing lesion or hidradenoma. It typically affects the face and scalp, although it can also cause nodal involvement and show up on the trunk, abdomen, or groin. Due to its similarities to other skin tumours, it is challenging to diagnose histopathologically. Therefore, immunohistochemical markers, such as p53, Ki-67, keratin AE1/AE3, and cytokeratin 5/6, are crucial for the diagnoses. There are no prospective randomized trials available to direct treatment of hidradenocarcinoma. A key component of therapy is early diagnoses and case discussion in multidisciplinary tumor board. Wide local excision with clear margins is the mainstay of treatment. Adjuvant treatment like chemo radiation therapy is incorporated if nodal disease or positive margins are present. Recurrences are treated by re-excision followed by adjuvant radiation therapy. Adjuvant hormonal therapy depends on the receptor status of the disease. These tumors have a 50% recurrence rate and up to 60% of the time they metastasize to the brain, bone, lung, liver, mediastinum, or peritoneum in a two-year period. The overall five year survival rate is around 30%. Keeping in view the aggressive course of this disease, research based clinically evident new treatment strategies are the need of time to optimize overall survival and local disease control. Keywords: Sweat gland tumors, Adnexal tumors, Hidradenocarcinoma, Adjuvant therapy, Multidisciplinary team, Radiation therapy.","PeriodicalId":500619,"journal":{"name":"National journal of health sciences","volume":"75 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2023-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"National journal of health sciences","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.21089/njhs.83.0128","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Hidradenocarcinoma makes up 6% of eccrine malignancies, and is an uncommon eccrine sweat gland tumour. The etiology is due to genetic alterations and transformation from an existing lesion or hidradenoma. It typically affects the face and scalp, although it can also cause nodal involvement and show up on the trunk, abdomen, or groin. Due to its similarities to other skin tumours, it is challenging to diagnose histopathologically. Therefore, immunohistochemical markers, such as p53, Ki-67, keratin AE1/AE3, and cytokeratin 5/6, are crucial for the diagnoses. There are no prospective randomized trials available to direct treatment of hidradenocarcinoma. A key component of therapy is early diagnoses and case discussion in multidisciplinary tumor board. Wide local excision with clear margins is the mainstay of treatment. Adjuvant treatment like chemo radiation therapy is incorporated if nodal disease or positive margins are present. Recurrences are treated by re-excision followed by adjuvant radiation therapy. Adjuvant hormonal therapy depends on the receptor status of the disease. These tumors have a 50% recurrence rate and up to 60% of the time they metastasize to the brain, bone, lung, liver, mediastinum, or peritoneum in a two-year period. The overall five year survival rate is around 30%. Keeping in view the aggressive course of this disease, research based clinically evident new treatment strategies are the need of time to optimize overall survival and local disease control. Keywords: Sweat gland tumors, Adnexal tumors, Hidradenocarcinoma, Adjuvant therapy, Multidisciplinary team, Radiation therapy.