Clinical Journal of Gastroenterology

, Volume 11, Issue 4, pp 333–337 | Cite as

Neuroendocrine neoplasm of pancreas with cystic degeneration mimicking mucinous cystic neoplasm

  • Uayporn Kaosombatwattana
  • Yoshiki Hirooka
  • Hiroki Kawashima
  • Eizaburo Ohno
  • Takuya Ishikawa
  • Hiroki Suhara
  • Hidemi Goto
Case Report


Endoscopic ultrasound is increasingly being used for evaluation of pancreatic diseases and pancreatic tumors. Among various pancreatic cystic lesions, cystic degeneration of pancreatic neuroendocrine neoplasm is of the challenge in making diagnosis. Although unique characteristic of each type of pancreatic cystic lesions has been proposed abundantly, typical morphology of cystic degeneration of pancreatic neuroendocrine neoplasm is still unclear. We, herein, reported a case of 66-year-old woman who was incidentally found to have a cystic lesion in the tail of pancreas upon screening transabdominal ultrasonography. A well-defined cystic lesion with rim calcification was noted on subsequent abdominal computed tomography. Endoscopic ultrasound revealed a markedly thick-wall cystic lesion containing solid nodule inside which was not enhanced following contrast-enhanced study. A mucinous cystic neoplasm was suspected and the patient was proceeded with distal pancreatectomy. A definite diagnosis of neuroendocrine neoplasm was confirmed after staining with synaptophysin and chromogranin A. We performed a meticulous review on current literatures focusing on endoscopic characteristics of pancreatic neuroendocrine neoplasms with cystic degeneration.


Pancreatic neuroendocrine neoplasm Cystic degeneration Pancreatic cystic lesion Endoscopic ultrasound Rim calcification 



The authors declare that they have no competing interests and no funding was received for this case report.

Compliance with ethical standards

Conflict of interest

All authors declare that they have no conflict of interest.

Human rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from the patient for being included in the report.

Supplementary material

12328_2018_846_MOESM1_ESM.mp4 (1.4 mb)
Supplementary material 1 VDO: Contrast-enhanced EUS first minute after injection with Sonazoid (MP4 1470 kb)


  1. 1.
    Baker MS, Knuth JL, DeWitt J, et al. Pancreatic cystic neuroendocrine tumors: preoperative diagnosis with endoscopic ultrasound and fine-needle immunocytology. J Gastrointest Surg. 2008;12:450–6.CrossRefPubMedGoogle Scholar
  2. 2.
    Figueiredo FA, Giovannini M, Monges G, et al. EUS-FNA predicts 5-year survival in pancreatic endocrine tumors. Gastrointest Endosc. 2009;70:907–14.CrossRefPubMedGoogle Scholar
  3. 3.
    Atiq M, Bhutani MS, Bektas M, et al. EUS-FNA for pancreatic neuroendocrine tumors: a tertiary cancer center experience. Dig Dis Sci. 2012;57:791–800.CrossRefPubMedGoogle Scholar
  4. 4.
    Bordeianou L, Vagefi PA, Sahani D, et al. Cystic pancreatic endocrine neoplasms: a distinct tumor type? J Am Coll Surg. 2008;206:1154–8.CrossRefPubMedGoogle Scholar
  5. 5.
    Yamada S, Fujii T, Suzuki K, et al. Preoperative identification of a prognostic factor for pancreatic neuroendocrine tumors using multiphase contrast-enhanced computed tomography. Pancreas. 2016;45:198–203.CrossRefPubMedGoogle Scholar
  6. 6.
    Ahrendt SA, Komorowski RA, Demeure MJ, et al. Cystic pancreatic neuroendocrine tumors: is preoperative diagnosis possible? J Gastrointest Surg. 2002;6:66–74.CrossRefPubMedGoogle Scholar
  7. 7.
    Buetow PC, Parrino TV, Buck JL, et al. Islet cell tumors of the pancreas: pathologic-imaging correlation among size, necrosis and cysts, calcification, malignant behavior, and functional status. AJR Am J Roentgenol. 1995;165:1175–9.CrossRefPubMedGoogle Scholar
  8. 8.
    Singhi AD, Chu LC, Tatsas AD, et al. Cystic pancreatic neuroendocrine tumors: a clinicopathologic study. Am J Surg Pathol. 2012;36:1666–73.CrossRefPubMedGoogle Scholar
  9. 9.
    Jiao Y, Shi C, Edil BH, et al. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011;331:1199–203.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Ridtitid W, Halawi H, DeWitt JM, et al. Cystic pancreatic neuroendocrine tumors: outcomes of preoperative endosonography-guided fine needle aspiration, and recurrence during long-term follow-up. Endoscopy. 2015;47:617–25.CrossRefPubMedGoogle Scholar
  11. 11.
    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(83–96):e2.Google Scholar
  12. 12.
    Cloyd JM, Kopecky KE, Norton JA, et al. Neuroendocrine tumors of the pancreas: degree of cystic component predicts prognosis. Surgery. 2016;160:708–13.CrossRefPubMedGoogle Scholar
  13. 13.
    Cho CS, Russ AJ, Loeffler AG, et al. Preoperative classification of pancreatic cystic neoplasms: the clinical significance of diagnostic inaccuracy. Ann Surg Oncol. 2013;20:3112–9.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Del Chiaro M, Segersvard R, Pozzi Mucelli R, et al. Comparison of preoperative conference-based diagnosis with histology of cystic tumors of the pancreas. Ann Surg Oncol. 2014;21:1539–44.CrossRefPubMedGoogle Scholar
  15. 15.
    Kawamoto S, Johnson PT, Shi C, et al. Pancreatic neuroendocrine tumor with cystlike changes: evaluation with MDCT. AJR Am J Roentgenol. 2013;200:W283–90.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Ring EJ, Eaton SB Jr, Ferrucci JT Jr, et al. Differential diagnosis of pancreatic calcification. Am J Roentgenol Radium Ther Nucl Med. 1973;117:446–52.CrossRefPubMedGoogle Scholar
  17. 17.
    Verde F, Fishman EK. Calcified pancreatic and peripancreatic neoplasms: spectrum of pathologies. Abdom Radiol (N Y). 2017;42:2686–97.CrossRefGoogle Scholar
  18. 18.
    Humphrey PE, Alessandrino F, Bellizzi AM, et al. Non-hyperfunctioning pancreatic endocrine tumors: multimodality imaging features with histopathological correlation. Abdom Imaging. 2015;40:2398–410.CrossRefPubMedGoogle Scholar
  19. 19.
    Li T, Chen ZQ, Meng ZX, et al. Calcified cystic lesion of the pancreas. J Gastrointest Surg. 2016;20:1272–4.CrossRefPubMedGoogle Scholar
  20. 20.
    Lesniak RJ, Hohenwalter MD, Taylor AJ. Spectrum of causes of pancreatic calcifications. AJR Am J Roentgenol. 2002;178:79–86.CrossRefPubMedGoogle Scholar
  21. 21.
    Kongkam P, Al-Haddad M, Attasaranya S, et al. EUS and clinical characteristics of cystic pancreatic neuroendocrine tumors. Endoscopy. 2008;40:602–5.CrossRefPubMedGoogle Scholar
  22. 22.
    Charfi S, Marcy M, Bories E, et al. Cystic pancreatic endocrine tumors: an endoscopic ultrasound-guided fine-needle aspiration biopsy study with histologic correlation. Cancer. 2009;117:203–10.PubMedGoogle Scholar
  23. 23.
    Colaiacovo R, de Castro AC, Ganc RL, et al. Cystic carcinoid tumor of the pancreas diagnosed by endoscopic ultrasound-guided fine needle aspiration of the cystic wall: an unusual presentation and diagnosis. Einstein (Sao Paulo). 2014;12:254–5.CrossRefGoogle Scholar
  24. 24.
    Thorlacius H, Kalaitzakis E, Johansson GW, et al. Cystic neuroendocrine tumor in the pancreas detected by endoscopic ultrasound and fine-needle aspiration: a case report. BMC Res Notes. 2014;7:510.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Yoon WJ, Daglilar ES, Pitman MB, et al. Cystic pancreatic neuroendocrine tumors: endoscopic ultrasound and fine-needle aspiration characteristics. Endoscopy. 2013;45:189–94.CrossRefPubMedGoogle Scholar
  26. 26.
    Vitali F, Strobel D, Pfeifer L, et al. Neuroendocrine tumor of the pancreas with cystic appearance mimicking a progressive intraductal papillary mucinous neoplasm: pitfall in medical imaging. Endoscopy. 2016;48:E302–3.CrossRefPubMedGoogle Scholar
  27. 27.
    Ishikawa T, Itoh A, Kawashima H, et al. Usefulness of EUS combined with contrast-enhancement in the differential diagnosis of malignant versus benign and preoperative localization of pancreatic endocrine tumors. Gastrointest Endosc. 2010;71:951–9.CrossRefPubMedGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  1. 1.Department of Gastroenterology and HepatologyNagoya University Graduate School of MedicineAichiJapan
  2. 2.Department of EndoscopyNagoya University HospitalNagoyaJapan
  3. 3.Department of Medicine, Faculty of Medicine Siriraj HospitalMahidol UniversityBangkokThailand

Personalised recommendations