RT Journal Article SR Electronic T1 Comprehensive characterisation of acinar cystic transformation of the pancreas: a systematic review JF Journal of Clinical Pathology JO J Clin Pathol FD BMJ Publishing Group Ltd and Association of Clinical Pathologists SP 740 OP 746 DO 10.1136/jcp-2023-209103 VO 76 IS 11 A1 Mattiolo, Paola A1 Wang, Huamin A1 Basturk, Olca A1 Brosens, Lodewijk A A A1 Hong, Seung-Mo A1 Adsay, Volkan A1 Scarpa, Aldo A1 Luchini, Claudio YR 2023 UL http://jcp.bmj.com/content/76/11/740.abstract AB Aims Acinar cystic transformation (ACT) of the pancreas is a rare pancreatic cystic lesion. Owing to its rarity, comprehensive histomolecular characterisation of this entity is still lacking. We aim to perform a systematic review on this controversial entity.Methods 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.Results 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 cm. 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 ≤3%. At the molecular level, three cases demonstrated genetic alterations: one showed multiple chromosomal gains, and other two harboured somatic mutations of KRAS and SMO genes (one mutation per case).Conclusions 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.All data relevant to the study are included in the article or uploaded as supplementary information.