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Owing to its rarity, comprehensive histomolecular characterisation of this entity is still lacking. We aim to perform a systematic review on this controversial entity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We searched PubMed, SCOPUS and Embase through May 2023 to identify all studies on ACTs. Clinicopathological, immunohistochemical (IHC) and molecular data have been extracted and analysed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Overall, there were 121 cases of ACTs in the literature. ACT had a female predominance (65.3% of patients), and a mean size of 4.8\u2009cm. ACT was more often unifocal (71.9%) and multiloculate (61.2%). Histologically, the cysts were lined by an acinar epithelium, sometimes harbouring ductal-like areas (18.2%). In five cases (4.1%), an intralesional pancreatic intraepithelial neoplasia (PanIN) was reported. Preoperative diagnosis is challenging. After surgical resection, all patients were alive and disease free during follow-up except one patient who developed a second ACT after resection. By IHC, all lesions were positive for acinar markers; cytokeratin 7 and 8\/18\/19 were usually positive, and Ki-67 was invariably \u22643%. At the molecular level, three cases demonstrated genetic alterations: one showed multiple chromosomal gains, and other two harboured somatic mutations of\n                      <jats:italic>KRAS<\/jats:italic>\n                      and\n                      <jats:italic>SMO<\/jats:italic>\n                      genes (one mutation per case).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Globally considered, our findings demonstrated that ACT is a benign entity, without the need of surgical resection with the exception of symptomatic lesions. The rare occurrence of intracystic PanINs and driver mutations suggest considering follow-up if a preoperative diagnosis of ACT can be made.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jcp-2023-209103","type":"journal-article","created":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T13:30:16Z","timestamp":1693315816000},"page":"740-746","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":10,"title":["Comprehensive characterisation of acinar cystic transformation of the pancreas: a systematic review"],"prefix":"10.1136","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5601-0879","authenticated-orcid":false,"given":"Paola","family":"Mattiolo","sequence":"first","affiliation":[{"name":"Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, 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