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  • Review Article
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Diagnosis and management of pancreatic cystic neoplasms: current evidence and guidelines

Nature Reviews Gastroenterology & Hepatology volume 16, pages 676–689 (2019)Cite this article

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Abstract

Pancreatic cystic neoplasms (PCN) are a heterogeneous group of pancreatic cysts that include intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, serous cystic neoplasms and other rare cystic lesions, all with different biological behaviours and variable risk of progression to malignancy. As more pancreatic cysts are incidentally discovered on routine cross-sectional imaging, optimal surveillance for patients with PCN is becoming an increasingly common clinical problem, highlighting the need to balance cancer prevention with the risk of (surgical) overtreatment. This Review summarizes the latest developments in the diagnosis and management of PCN, including the quality of available evidence. Also discussed are the most important differences between the PCN guidelines from the American Gastroenterological Association, the International Association of Pancreatology and the European Study Group on Cystic Tumours of the Pancreas, including diagnostic and follow-up strategies and indications for surgery. Finally, new developments in the management of patients with PCN are addressed.

Key points

  • Pancreatic cysts are increasingly diagnosed and, although most are benign, some can develop into pancreatic cancer; uniform guidelines for diagnosis, treatment and follow-up of pancreatic cysts are therefore urgently required.

  • In revised guidelines, obstructive jaundice, a contrast-enhanced mural nodule or solid component ≥5 mm, a dilated pancreatic duct or positive cytology for advanced neoplasia are absolute indications for resection in patients with intraductal papillary mucinous neoplasms (IPMN).

  • In European guidelines, both a mucinous cystic neoplasm (MCN) and IPMN <40 mm are treated conservatively when other risk factors are absent.

  • In international and American guidelines, an MCN of any size is an absolute indication for resection; in the international guidelines, an IPMN >30 mm is a relative indication for resection.

  • Lifelong surveillance is indicated for patients with IPMN and MCN without risk factors, as long as they are fit and willing to undergo surgery.

  • Follow-up for IPMN after pancreatectomy is warranted because of the risk of developing recurrent disease, although evidence on the best surveillance interval is lacking.

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Fig. 1: Examples of different types of PCN.
Fig. 2: Contrast-enhanced EUS for discrimination between mural nodules and mucin clots.
Fig. 3: Surgical options for PCN.

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Author notes

  1. These authors contributed equally: Jeanin E van Hooft, Marc G Besselink.

Authors and Affiliations

  1. Department of Gastroenterology and Hepatology, Amsterdam Gastroenterology and Metabolism, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands

    Nadine C. M. van Huijgevoort & Jeanin E. van Hooft

  2. Department of Surgery, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA

    Marco del Chiaro

  3. Department of Surgery, The Sol Goldman Pancreatic Cancer Research Center, The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Christopher L. Wolfgang

  4. Department of Surgery, Cancer Center Amsterdam, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands

    Marc G. Besselink

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Contributions

All authors researched data for the article and provided a substantial contribution to discussion of the content. N.C.M.v.H. wrote the article. M.d.C., C.L.W., J.E.v.H. and M.G.B. contributed to reviewing and editing the manuscript before submission.

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Review criteria

We searched PubMed using the terms “pancreatic cystic neoplasm” combined with “classification”, “diagnosis”, “endoscopic ultrasound”, “cyst fluid analysis”, “guidelines”, “treatment”, “surgery”, “recurrence” and “surveillance”. We selected full-text articles in English published in the previous 10 years, but exceptions were made for older, highly cited papers. We aimed to describe the results of prospective studies, since randomized controlled trials are lacking in this field, but other studies are also referenced. In addition, references list of the finally included articles were checked manually for studies that had not been identified by the primary search. Some recommendations are based on the recently revised guidelines of the International Association of Pancreatology3 and the European Study Group on Cystic Tumours of the Pancreas4.

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van Huijgevoort, N.C.M., del Chiaro, M., Wolfgang, C.L. et al. Diagnosis and management of pancreatic cystic neoplasms: current evidence and guidelines. Nat Rev Gastroenterol Hepatol 16, 676–689 (2019). https://doi.org/10.1038/s41575-019-0195-x

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