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Their radiological features often overlap with malignant tumors, therefore ruling out malignancy becomes mandatory. We analyzed clinical, radiological, and histopathological data to identify specific characteristics of these tumors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed 21 patients with ACHs (n\u2009=\u200912), ACLs (n\u2009=\u20098), or adrenal cysts (n\u2009=\u20091) confirmed by histopathology. We selected 82 papers from PubMed to provide a systematic review of the literature.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In our cohort, median age at diagnosis was 58 years, with sex evenly distributed. All tumors were unilateral (median size\u2009=\u200944\u2009mm), with 6 cases of increasing tumor size. All tumors exhibited non-contrast CT density\u2009&gt;\u200910 Hounsfield Unit (HU). Calcifications were found in 5 cases. Hormonal studies revealed 11 non-functioning tumors and 2 cortisol-secreting tumors. Elevated urinary metanephrines were found in 2 cases. Immunostaining showed CD31\/CD34\/factor VIII expression in ACHs (n\u2009=\u20095, 24%) and podoplanin expression in ACLs (n\u2009=\u20096, 29%). The literature review revealed 71 reported cases of ACHs and 104 reported cases of ACLs. Median age at diagnosis was 46 years, with slightly female prevalence (63%). Median tumor size was 48\u2009mm. 84 cases were symptomatic, with life-threatening hemorrhage reported in only 3 patients. Calcifications were found in 23% of cases. Surgical approaches varied, with open and laparoscopic adrenalectomy performed in 55 and 42 patients respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>ACHs and ACLs represent a diagnostic dilemma in clinical practice due to their rarity and their misleading imaging features.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s12020-024-04123-5","type":"journal-article","created":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T19:37:20Z","timestamp":1737142640000},"page":"1291-1304","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["The diagnostic dilemma of adrenal vascular tumors: analysis of 21 cases and systematic review of the literature"],"prefix":"10.1007","volume":"87","author":[{"given":"Kimberly","family":"Coscia","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caterina","family":"Ravaioli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Tucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo","family":"Colombin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Donnarumma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Mosconi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caterina","family":"Balacchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Nanni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Alberici","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saverio","family":"Selva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uberto","family":"Pagotto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donatella","family":"Santini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Tallini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guido","family":"Di Dalmazi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Vicennati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"De Leo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,1,18]]},"reference":[{"issue":"7","key":"4123_CR1","doi-asserted-by":"publisher","first-page":"398","DOI":"10.1038\/s41574-023-00835-2","volume":"19","author":"J Calissendorff","year":"2023","unstructured":"J. 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