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These may arise from adrenal rest anywhere in the embryonic pathway of the adrenal glands. In the context of extra-adrenal and retroperitoneal tumors, the exact pathologic diagnosis is challenging. The case reported here, to the best of our knowledge, is the seventh reported case of extra-adrenal non-functional adrenocortical carcinoma.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>We report a case of extra-adrenal non-functional adrenocortical carcinoma in a 15-year-old Persian boy who presented with an acute abdomen. He underwent surgical resection. Pathologic findings based on immunohistochemistry and cellular morphology confirmed adrenocortical carcinoma. He was treated with mitotane for 24\u2009months. During a follow-up period of 30\u2009months, no recurrence or metastases were found.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>Despite the rarity of extra-adrenal adrenocortical carcinoma, presentation with an acute abdomen may occur, and the tumor may be found anywhere in the adrenal embryonic pathway. On the other hand, tumor behavior and prognosis in children may be different from what we expect in adults.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s13256-020-02408-7","type":"journal-article","created":{"date-parts":[[2020,7,7]],"date-time":"2020-07-07T23:06:39Z","timestamp":1594163199000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report"],"prefix":"10.1186","volume":"14","author":[{"given":"Alireza","family":"Mirsharifi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad","family":"Vasei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ehsan","family":"Sadeghian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Ghorbani-Abdehgah","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Naybandi Atashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,7,8]]},"reference":[{"issue":"5","key":"2408_CR1","doi-asserted-by":"publisher","first-page":"1255","DOI":"10.1016\/j.suc.2009.06.019","volume":"89","author":"M Wandoloski","year":"2009","unstructured":"Wandoloski M, Bussey KJ, Demeure MJ. 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A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests in any part from the treatment process to writing the manuscript. The chemotherapy agent \u2013 mitotane \u2013 may raise suspicion of conflict of interest, but we declare we have no conflict of interest with the company producing this medication, and this medication is the standard of treatment in this case and its use is inevitable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"107"}}