{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T05:08:32Z","timestamp":1767848912550,"version":"3.49.0"},"reference-count":16,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T00:00:00Z","timestamp":1724716800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Surgical Oncology"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Objectives<\/jats:title><jats:p>Primary adrenal sarcoma (PAS) is an exceedingly rare malignancy with limited data available on its epidemiology, management, and outcomes. This study aimed to characterize the national incidence, treatment patterns, and survival of PAS utilizing a National Cancer Database.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The National Cancer Database was queried for patients diagnosed with primary adrenal tumors from 2004 to 2019. Cases with sarcoma histology were identified as PAS. Annual incidence trends, histological distribution, treatment modalities (surgery, chemotherapy, radiation therapy), perioperative outcomes, and overall survival (OS) were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 7213 primary adrenal tumor cases, 332 (4.6%) were PAS. The most common histological subtypes were leiomyosarcoma (37.3%), hemangiosarcoma (27.1%), and sarcoma not otherwise specified (6.0%). Most cases (71.7%) presented as locoregional disease. Treatment included surgery alone (47.8%), surgery plus chemotherapy and\/or radiation (27.1%), chemotherapy\/radiation alone (13.3%), or no treatment (13.9%). For surgical cases, the median length of stay was 5 days, the 30\u2010day readmission rate was 3.36%, and the 30\/90\u2010day mortality rates were 3.65% and 9.90%, respectively. The 5\u2010year OS rate for surgery alone was 43%, with a median OS of 34.6 months. For surgery with radiation\/chemotherapy, the 5\u2010year OS rate was 37.3%, with a median OS of 35.4 months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This largest analysis of PAS to date demonstrates that most cases present as locoregional disease amenable to surgical resection, with favorable outcomes. The role of adjuvant therapy remains unclear, as no significant survival difference was observed between surgery alone and multimodal treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jso.27836","type":"journal-article","created":{"date-parts":[[2024,8,27]],"date-time":"2024-08-27T15:59:36Z","timestamp":1724774376000},"page":"1700-1705","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Primary adrenal sarcomas: A national analysis of epidemiological trends, treatment patterns, and outcomes"],"prefix":"10.1002","volume":"130","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0257-7439","authenticated-orcid":false,"given":"Bibek","family":"Aryal","sequence":"first","affiliation":[{"name":"Allegheny Singer Research Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samantha","family":"Falls","sequence":"additional","affiliation":[{"name":"Surgery Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yue","family":"Yin","sequence":"additional","affiliation":[{"name":"Allegheny Singer Research Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick L.","family":"Wagner","sequence":"additional","affiliation":[{"name":"Surgery Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David L.","family":"Bartlett","sequence":"additional","affiliation":[{"name":"Cancer Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rodney E.","family":"Wegner","sequence":"additional","affiliation":[{"name":"Cancer Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Casey J.","family":"Allen","sequence":"additional","affiliation":[{"name":"Surgery Institute Allegheny Health Network Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,8,27]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.4081\/cp.2014.604"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1530\/EC-18-0304"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2018-00918"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/20363613211057746"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijscr.2021.106707"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12902-023-01530-z"},{"key":"e_1_2_9_8_1","unstructured":"Surgeons. 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Cancer Programs National Cancer Database.2024.https:\/\/www.facs.org\/quality-programs\/cancer-programs\/national-cancer-database\/"},{"key":"e_1_2_9_9_1","volume-title":"International classification of diseases for oncology","author":"Fritz A","year":"2000"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-40618-3"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/CCO.0b013e32834796e6"},{"issue":"4","key":"e_1_2_9_12_1","first-page":"4258","article-title":"Primary adrenal leiomyosarcoma: a case report and review of literature","volume":"8","author":"Zhou Y","year":"2015","journal-title":"Int J Clin Exp Pathol"},{"issue":"6","key":"e_1_2_9_13_1","first-page":"481","article-title":"Surgery of primary unilateral adrenal gland tumors\u2010\u2010results of 154 patients","volume":"122","author":"Walz MK","year":"1997","journal-title":"Zentralbl Chir"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.4183\/aeb.2020.208"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1530\/EJE-18-0608"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2002.20.4.941"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.21037\/tlcr.2019.05.06"}],"container-title":["Journal of Surgical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jso.27836","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T07:57:28Z","timestamp":1741766248000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jso.27836"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,27]]},"references-count":16,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2024,12]]}},"alternative-id":["10.1002\/jso.27836"],"URL":"https:\/\/doi.org\/10.1002\/jso.27836","archive":["Portico"],"relation":{},"ISSN":["0022-4790","1096-9098"],"issn-type":[{"value":"0022-4790","type":"print"},{"value":"1096-9098","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,27]]},"assertion":[{"value":"2024-07-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-08-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}