Vol 69, No 3 (2018)
Review paper
Published online: 2018-06-25

open access

Page views 2847
Article views/downloads 1872
Get Citation

Connect on Social Media

Connect on Social Media

Cystic pancreatic neuroendocrine tumours — a gastroenterologist’s point of view

Krzysztof Dąbkowski1, Beata Kos-Kudła2, Elżbieta Andrysiak-Mamos3, Anhelli Syrenicz3, Joanna Pilch-Kowalczyk4, Teresa Starzyńska1
Pubmed: 29952422
Endokrynol Pol 2018;69(3):320-325.


Cystic pancreatic tumors are detected with increasing frequency and remain a clinical problem. Since they have different potential of malignancy the management and decision making process is a hard task. Guidelines, concerning pancreatic cystic tumors indicate the management with mucinous, serous cystic pancreatic neoplasms and solid pseudopappilary tumor, while the management with pancreatic cystic neuroendocrine tumors is not included into these standards. This review tries to answer the question are the cystic pancreatic neuroendocrine tumors different entity from solid tumors of neuroendocrine origin.The management and differential diagnosis of these neoplasms with special focus on features on imaging studies allowing preoperative diagnosis are discussed.

Article available in PDF format

View PDF Download PDF file


  1. Kapoor VK. Complications of pancreato-duodenectomy. Rozhl Chir. 2016; 95(2): 53–59.
  2. He J, Ahuja N, Makary MA, et al. 2564 resected periampullary adenocarcinomas at a single institution: trends over three decades. HPB (Oxford). 2014; 16(1): 83–90.
  3. Cameron JL, He J. Two thousand consecutive pancreaticoduodenectomies. J Am Coll Surg. 2015; 220(4): 530–536.
  4. Tanaka M, Fernández-Del Castillo C, Kamisawa T, et al. Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas. Pancreatology. 2017; 17(5): 738–753.
  5. Del Chiaro M, Verbeke C, Salvia R, et al. European Study Group on Cystic Tumours of the Pancreas. European experts consensus statement on cystic tumours of the pancreas. Dig Liver Dis. 2013; 45(9): 703–711.
  6. Singhi AD, Chu LC, Tatsas AD, et al. Cystic pancreatic neuroendocrine tumors: a clinicopathologic study. Am J Surg Pathol. 2012; 36(11): 1666–1673.
  7. Kos-Kudła B, Rosiek V, Borowska M, et al. Pancreatic neuroendocrine neoplasms - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017; 68(2): 169–197.
  8. Falconi M, Eriksson B, Kaltsas G, et al. Vienna Consensus Conference participants. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology. 2016; 103(2): 153–171.
  9. Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J, et al. Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017; 68(2): 79–110.
  10. Zhang XM, Mitchell DG, Dohke M, et al. Pancreatic cysts: depiction on single-shot fast spin-echo MR images. Radiology. 2002; 223(2): 547–553.
  11. Spinelli K, Fromwiller T, Daniel R, et al. Cystic pancreatic neoplasms: observe or operate. Ann Surg. 2004; 239(5): 651–659.
  12. Laffan TA, Horton KM, Klein AP, et al. Prevalence of unsuspected pancreatic cysts on MDCT. AJR Am J Roentgenol. 2008; 191(3): 802–807.
  13. Kimura W, Nagai H, Kuroda A, et al. Analysis of small cystic lesions of the pancreas. Int J Pancreatol. 1995; 18(3): 197–206.
  14. Dabkowski K, Kojder K, Smereczyński A, et al. [Rare solid pancreatic tumors]. Pol Merkur Lekarski. 2013; 35(206): 111–118.
  15. Dabkowski K, Kojder K, Smereczyński A, et al. [Rare solid pancreatic tumors]. Pol Merkur Lekarski. 2013; 35(206): 111–118.
  16. Caglià P, Cannizzaro MT, Tracia A, et al. Cystic pancreatic neuroendocrine tumors: To date a diagnostic challenge. Int J Surg. 2015; 21 Suppl 1: S44–S49.
  17. Koh YX, Chok AY, Zheng HL, et al. A systematic review and meta-analysis of the clinicopathologic characteristics of cystic versus solid pancreatic neuroendocrine neoplasms. Surgery. 2014; 156(1): 83–96.e2.
  18. Bordeianou L, Vagefi PA, Sahani D, et al. Cystic pancreatic endocrine neoplasms: a distinct tumor type? J Am Coll Surg. 2008; 206(6): 1154–1158.
  19. Morales-Oyarvide V, Yoon WJ, Ingkakul T, et al. Cystic pancreatic neuroendocrine tumors: the value of cytology in preoperative diagnosis. Cancer Cytopathol. 2014; 122(6): 435–444.
  20. Gaujoux S, Tang L, Klimstra D, et al. The outcome of resected cystic pancreatic endocrine neoplasms: a case-matched analysis. Surgery. 2012; 151(4): 518–525.
  21. Sureka B, Bihari C, Arora A, et al. Imaging paradigm of cystic lesions in pancreas. JOP Journal of the Pancreas. 2016; 17(5).
  22. Hurtado-Pardo L, A Cienfuegos J, Ruiz-Canela M, et al. Cystic pancreatic neuroendocrine tumors (cPNETs): a systematic review and meta-analysis of case series. Rev Esp Enferm Dig. 2017; 109(11): 778–787.
  23. Buetow PC, Miller DL, Parrino TV, et al. Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. Radiographics. 1997; 17(2): 453–72; quiz 472A.
  24. Cloyd JM, Kopecky KE, Norton JA, et al. Neuroendocrine tumors of the pancreas: Degree of cystic component predicts prognosis. Surgery. 2016; 160(3): 708–713.
  25. Paiella S, Marchegiani G, Miotto M, et al. Are Cystic Pancreatic Neuroendocrine Tumors an Indolent Entity Results from a Single-Center Surgical Series. Neuroendocrinology. 2018; 106(3): 234–241.
  26. Yoon WJ, Daglilar ES, Pitman MB, et al. Cystic pancreatic neuroendocrine tumors: endoscopic ultrasound and fine-needle aspiration characteristics. Endoscopy. 2013; 45(3): 189–194.
  27. Koniusz J, Dąbkowski K, Buczek K, et al. [Gastroenterological manifestations of von Hippel-Lindau disease]. Pol Merkur Lekarski. 2017; 43(254): 53–55.
  28. Dumonceau JM, Deprez PH, Jenssen C, et al. Indications, results, and clinical impact of endoscopic ultrasound (EUS)-guided sampling in gastroenterology: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline - Updated January 2017. Endoscopy. 2017; 49(7): 695–714.
  29. de Jong K, Poley JW, van Hooft JE, et al. Endoscopic ultrasound-guided fine-needle aspiration of pancreatic cystic lesions provides inadequate material for cytology and laboratory analysis: initial results from a prospective study. Endoscopy. 2011; 43(7): 585–590.
  30. Thosani N, Thosani S, Qiao W, et al. Role of EUS-FNA-based cytology in the diagnosis of mucinous pancreatic cystic lesions: a systematic review and meta-analysis. Dig Dis Sci. 2010; 55(10): 2756–2766.
  31. Giuliano K, Ejaz A, He J. Technical aspects of pancreaticoduodenectomy and their outcomes. Chin Clin Oncol. 2017; 6(6): 64.
  32. Kawamoto S, Johnson PT, Shi C, et al. Pancreatic neuroendocrine tumor with cystlike changes: evaluation with MDCT. AJR Am J Roentgenol. 2013; 200(3): W283–W290.
  33. Ahrendt SA, Komorowski RA, Demeure MJ, et al. Cystic pancreatic neuroendocrine tumors: is preoperative diagnosis possible? J Gastrointest Surg. 2002; 6(1): 66–74.
  34. Khashab MA, Kim K, Lennon AM, et al. Should we do EUS/FNA on patients with pancreatic cysts? The incremental diagnostic yield of EUS over CT/MRI for prediction of cystic neoplasms. Pancreas. 2013; 42(4): 717–721.
  35. Kongkam P, Al-Haddad M, Attasaranya S, et al. EUS and clinical characteristics of cystic pancreatic neuroendocrine tumors. Endoscopy. 2008; 40(7): 602–605.
  36. Lakhtakia S. Complications of diagnostic and therapeutic Endoscopic Ultrasound. Best Pract Res Clin Gastroenterol. 2016; 30(5): 807–823.
  37. Kishida Y, Matsubayashi H, Okamura Y, et al. A case of solid-type serous cystadenoma mimicking neuroendocrine tumor of the pancreas. J Dig Dis. 2014; 15(4): 211–215.
  38. Oruç N, Aydın A, Barutcuoğlu B, et al. Cystic fluid chromogranin A levels in different pancreatic cystic lesions. Turk J Gastroenterol. 2015; 26(6): 522–527.
  39. Dabkowski K, Lubikowski J, Głuszko R, et al. [Pancreatic solid pseudopapillary tumor — report of three cases]. Pol Merkur Lekarski. 2014; 36(214): 265–269.