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We conducted a retrospective single-institution cohort study of 65 resected primary ACCs from 2010 to 2025. Clinicopathologic features, tumor-type-specific histologic scoring, immunohistochemistry for p53, \u03b2-catenin, and Ki-67, clinical molecular testing results, and outcomes were analyzed. Survival was evaluated using Kaplan\u2013Meier methods and Cox models adjusted for pT category. Increased mitotic activity (&gt;\u20095 mitoses per 50 high-power fields) was present in 41\/55 tumors (74.5%). Clinical molecular testing was performed in 32 tumors; 12 (37.5%) had at least one pathogenic or likely pathogenic (P\/LP) alteration, most commonly involving TP53 (6\/12) and CTNNB1 (3\/12). P\/LP alteration positivity was associated with worse overall survival (OS) after pT adjustment (HR, 4.67; 95% CI, 1.26\u201317.26; p\u2009=\u20090.021). Nuclear \u03b2-catenin expression was also associated with worse OS after pT adjustment (HR, 3.23; 95% CI, 1.19\u20138.79; p\u2009=\u20090.022). Increased mitotic activity remained associated with worse OS after pT adjustment (HR, 4.62; 95% CI, 1.07\u201319.91; p\u2009=\u20090.040), whereas its association with disease-specific survival did not reach statistical significance after pT adjustment (HR, 7.44; 95% CI, 0.98\u201356.21; p\u2009=\u20090.052). Ki-67\u2009\u2265\u200910%, aberrant p53 staining, tumor necrosis, and angioinvasion were not significantly associated with survival after pT adjustment. Oncocytic ACCs had less frequent increased mitotic activity than conventional ACCs (44.4% vs. 79.1%; p\u2009=\u20090.048). Mitotic activity, nuclear \u03b2-catenin expression, and clinically reported P\/LP alterations may provide complementary prognostic information in resected primary ACC. Larger cohorts with standardized pathology review and uniform molecular profiling are needed.<\/jats:p>","DOI":"10.1007\/s00428-026-04734-5","type":"journal-article","created":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T05:09:16Z","timestamp":1790572156000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinicopathologic, immunohistochemical, and molecular correlates in resected primary adrenocortical carcinoma: a single-institution cohort study"],"prefix":"10.1007","author":[{"given":"Wei","family":"Du","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8868-9700","authenticated-orcid":false,"given":"Shunsuke","family":"Koga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selemon","family":"Walle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lin","family":"Mei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zubair","family":"Baloch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,28]]},"reference":[{"key":"4734_CR1","doi-asserted-by":"publisher","first-page":"2579","DOI":"10.1016\/j.ejca.2013.02.034","volume":"49","author":"TM Kerkhofs","year":"2013","unstructured":"Kerkhofs TM, Verhoeven RH, Van der Zwan JM, Dieleman J, Kerstens MN, Links TP, Van de Poll-Franse LV, Haak HR (2013) Adrenocortical carcinoma: a population-based study on incidence and survival in the Netherlands since 1993. 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