{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T02:15:00Z","timestamp":1791598500963,"version":"4.3.4"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,4,22]],"date-time":"2021-04-22T00:00:00Z","timestamp":1619049600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100016144","name":"Fondazione Internazionale di Ricerca in Medicina Onlus","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100016144","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005010","name":"Associazione Italiana per la Ricerca sul Cancro","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005010","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>In a recently published retrospective case series, Temozolomide was found active as second line approach in advanced ACC patients. The disease control rate obtained, however, was short-lived. We report here an ACC patient with extensive metastatic disease who obtained a remarkable long lasting response with this alkylating agent.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>a 22-year-old female patient with ACC presented at our Medical Oncology Department in poor general condition due the presence of extensive metastatic pulmonary involvement. The disease had progressed to etoposide, doxorubicin and cisplatin plus mitotane therapy. Second line temozolomide therapy was prescribed leading to a progressive improvement of patient general conditions. The disease restaging after 12 cycles revealed a complete response of lung lesions and the patient was free from progression for 14+ months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Temozolomide therapy could be exceptionally efficacious in the management of ACC patients. The molecular mechanisms of sensitivity and resistance to this drug should be carefully studied, in order to select the patients destined to obtain a significant clinical benefit to the drug.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fendo.2021.674039","type":"journal-article","created":{"date-parts":[[2021,4,22]],"date-time":"2021-04-22T13:12:45Z","timestamp":1619097165000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":7,"title":["Case Report: Exceptional Response to Second Line Temozolomide Therapy in a Patient With Metastatic Adrenocortical Carcinoma"],"prefix":"10.3389","volume":"12","author":[{"given":"Deborah","family":"Cosentini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Turla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ornella","family":"Carminati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salvatore","family":"Grisanti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittorio Domenico","family":"Ferrari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Lagan\u00e0","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Rosti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Sigala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfredo","family":"Berruti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,4,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.annonc.2020.08.2099","article-title":"Adrenocortical Carcinomas and Malignant Phaeochromocytomas: ESMO-EURACAN Clinical Practice Guidelines for Diagnosis, Treatment and Follow-Up","volume":"31","author":"Fassnacht","year":"2020","journal-title":"Ann Oncol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2016-2894","article-title":"Long-Term Outcomes of Adjuvant Mitotane Therapy in Patients With Radically Resected Adrenocortical Carcinoma","volume":"102","author":"Berruti","year":"2017","journal-title":"J Clin Endocrinol Metab"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1677\/erc.1.01025","article-title":"Etoposide, Doxorubicin and Cisplatin Plus Mitotane in the Treatment of Advanced Adrenocortical Carcinoma: A Large Prospective Phase II Trial","volume":"12","author":"Berruti","year":"2005","journal-title":"Endocr Relat Cancer"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1200966","article-title":"Combination Chemotherapy in Advanced Adrenocortical Carcinoma","volume":"366","author":"Fassnacht","year":"2012","journal-title":"N Engl J Med"},{"key":"B5","doi-asserted-by":"publisher","first-page":"941","DOI":"10.3390\/cancers12040941","article-title":"Efficacy of the EDP-M Scheme Plus Adjunctive Surgery in the Management of Patients With Advanced Adrenocortical Carcinoma: The Brescia Experience","volume":"12","author":"Lagan\u00e0","year":"2020","journal-title":"Cancers (Basel)"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1677\/ERC-09-0281","article-title":"Gemcitabine Plus Metronomic 5-Fluorouracil or Capecitabine as a Second-\/Third-Line Chemotherapy in Advanced Adrenocortical Carcinoma: A Multicenter Phase II Study","volume":"17","author":"Sperone","year":"2010","journal-title":"Endocr Relat Cancer"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s40618-013-0049-2","article-title":"Management of Adrenal Cancer: A 2013 Update","volume":"37","author":"Terzolo","year":"2014","journal-title":"J Endocrinol Invest"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.coemr.2019.07.007","article-title":"Are We Failing in Treatment of Adrenocortical Carcinoma? 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