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Comprehensive characterisation of acinar cystic transformation of the pancreas: a systematic review
  1. Paola Mattiolo1,
  2. Huamin Wang2,
  3. Olca Basturk3,
  4. Lodewijk A A Brosens4,
  5. Seung-Mo Hong5,
  6. Volkan Adsay6,
  7. Aldo Scarpa7,
  8. Claudio Luchini7
  1. 1 Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona, Italy
  2. 2 Department of Anatomical Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
  3. 3 Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
  4. 4 Department of Pathology, Utrecht University, Utrecht, The Netherlands
  5. 5 Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea (the Republic of)
  6. 6 Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), Istanbul, Turkey
  7. 7 Department of Diagnostics and Public Health, Section of Pathology, and ARC-Net Research Center, University and Hospital Trust of Verona, Verona, Italy
  1. Correspondence to Professor Claudio Luchini, Diagnostics and Public Health, Universita degli Studi di Verona, Verona, Italy; claudio.luchini{at}


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.

  • Pancreatic Neoplasms
  • Morphological and Microscopic Findings
  • Pathology, Molecular

Data availability statement

All data relevant to the study are included in the article or uploaded as supplementary information.

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Data availability statement

All data relevant to the study are included in the article or uploaded as supplementary information.

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  • Handling editor Yoh Zen.

  • Twitter @paolamattiolo, @OlcaBasturk, @CLuchini10

  • Collaborators No.

  • Contributors PM and CL: study conception and design; PM, HW, AS and CL: systematic review; all authors: data discussion and interpretation; PM and CL: paper writing; all authors: final editing and approval of the final version. CL: guarantor of the overall content.

  • Funding This study is supported by Associazione Italiana Ricerca sul Cancro (AIRC IG n. 26343); Fondazione Cariverona: Oncology Biobank Project 'Antonio Schiavi' (prot. 203885/2017); Fondazione Italiana Malattie Pancreas (FIMP-Ministero Salute J38D19000690001); personal university-funds for research (FUR Luchini, Verona University).

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.