Comprehensive characterisation of acinar cystic transformation of the pancreas: a systematic review

Abstract

AimsAcinar 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.MethodsWe 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.ResultsOverall, 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 ofKRASandSMOgenes (one mutation per case).ConclusionsGlobally 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.

Familial Versus Nonfamilial Origin of Acinar Cystic Transformation of the Pancreas: An Important Question to Address

“…Nevertheless, the authors reported reliable radiological evidences supporting the diagnosis. Interestingly, all cases of ACT described in their article showed calcifications, an uncommon finding in ACT as shown by a recent systematic review on this topic 3 . Of note, their findings provide new evidences for familial aggregation of ACT.…”

“…Interestingly, all cases of ACT described in their article showed calcifications, an uncommon finding in ACT as shown by a recent systematic review on this topic. 3 Of note, their findings provide new evidences for familial aggregation of ACT. Along this line, a recent investigation presenting the largest series of ACTs in the literature has highlighted the potential existence of different and heterogeneous etiopathologies for this entity.…”