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Given the paucity of cases in the literature, there is a lack of consensus on treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The terms \u2018malignant spiradenoma\u2019 or \u2018spiradenocarcinoma\u2019 were systematically used to search the PubMed, MEDLINE and Google Scholar databases. A total of 182 cases of spiradenocarcinoma were identified as eligible for this comprehensive literature review.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Spiradenocarcinoma was commoner in older age and Caucasian race. In most cases, surgical excision for local disease is the mainstay of treatment. Lymph node dissection is usually reserved for those with suspected or confirmed lymph node metastases. High rates of local recurrence (20.8%), metastasis (37.4%) and mortality (19.1%) were identified, prompting some authors to suggest regular follow up including chest X\u2010rays and liver function tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with spiradenocarcinoma may benefit from a magnetic resonance imaging and fluorodeoxyglucose\u2010positron emission tomography\/computed tomography to establish the extent of disease. We recommend wide local excision as the treatment of choice to achieve surgical margins of \u22651\u00a0cm, with node resection to be determined on a case\u2010to\u2010case basis. Regular follow up is important given the high rate of local recurrence, metastasis and mortality. This should include an examination of the regional lymph nodes. Further research is required to refine an evidence\u2010based approach to spiradenocarcinoma.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ans.16626","type":"journal-article","created":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T04:22:11Z","timestamp":1612239731000},"page":"1996-2001","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Challenges in diagnosis and management of a spiradenocarcinoma: a comprehensive literature review"],"prefix":"10.1111","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8657-0164","authenticated-orcid":false,"given":"Katy","family":"Wagner","sequence":"first","affiliation":[{"name":"Monash University Endocrine Surgery Unit Alfred Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karishma","family":"Jassal","sequence":"additional","affiliation":[{"name":"Monash University Endocrine Surgery Unit Alfred Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James C.","family":"Lee","sequence":"additional","affiliation":[{"name":"Monash University Endocrine Surgery Unit Alfred Hospital  Melbourne Victoria Australia"},{"name":"Department of Surgery Central Clinical School, Monash University, The Alfred Centre  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ee\u2010Jun","family":"Ban","sequence":"additional","affiliation":[{"name":"Monash University Endocrine Surgery Unit Alfred Hospital  Melbourne Victoria Australia"},{"name":"Department of Surgery Central Clinical School, Monash University, The Alfred Centre  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhoda","family":"Cameron","sequence":"additional","affiliation":[{"name":"Department of Pathology Alfred Hospital  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan","family":"Serpell","sequence":"additional","affiliation":[{"name":"Monash University Endocrine Surgery Unit Alfred Hospital  Melbourne Victoria Australia"},{"name":"Department of Surgery Central Clinical School, Monash University, The Alfred Centre  Melbourne Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,2]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"388","article-title":"Malignant transformation of eccrine spiradenoma","volume":"11","author":"Dabska M","year":"1972","journal-title":"Pol. 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