Skip to main content

Advertisement

Log in

A case of neuroendocrine neoplasm of the minor duodenal papilla

  • Case Report
  • Published:
Clinical Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

A 61-year-old woman was referred to our hospital for intraductal papillary mucinous neoplasm with no symptoms. Magnetic resonance imaging (MRI) depicted a 15 mm nodular lesion at the descending portion of duodenum. Upper gastrointestinal endoscopy showed a submucosal tumor-like mass at the minor duodenal papilla. A boring biopsy of the tumor revealed a neuroendocrine neoplasm (NEN). Various blood hormone tests were all within normal limits, and the tumor was considered to be nonfunctional. Contrast-enhanced computed tomography showed no obvious distant metastasis, and subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) was performed. Histopathological examination revealed a dense cluster of spindle-shaped cells forming a sheet-like foci and areas showing glandular lumen formation, and immunohistochemistry showed synaptophysin ( + ), chromogranin ( + ). Mitotic rate was about 11 mitoses per 2 square millimeters, Ki-67 index was about 0.2%. She was pathologically diagnosed with NEN G2 at the minor duodenal papilla with regional lymph node metastasis according to the WHO2010 classification. The patient has been currently under outpatient observation with a good postoperative course. Review of the literature identified 43 cases of NENs of the minor duodenal papilla. NENs of the minor duodenal papilla have a high rate of lymph node metastasis, even if the tumor size is small.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

Data availability

Data sharing is not applicable to this article as no new data were created or analyzed in this study.

Abbreviations

CT:

Computed tomography

MRI:

Magnetic resonance imaging

EUS:

Endoscopic ultrasonography

IPMN:

Intraductal papillary mucinous neoplasm

NEN:

Neuroendocrine neoplasm

References

  1. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97:934–59.

    Article  PubMed  Google Scholar 

  2. Ito T, Igarashi H, Nakamura K, et al. Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis. J Gastroenterol. 2015;50:58–64.

    Article  PubMed  Google Scholar 

  3. Ito T, Sasano H, Tanaka M, et al. Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan. J Gastroenterol. 2010;45:234–43.

    Article  PubMed  Google Scholar 

  4. Soga J. Endocrinocarcinomas (carcinoids and their variants) of the duodenum. An evaluation of 927 cases. J Exp Clin Cancer Res. 2003;22:349–63.

    CAS  PubMed  Google Scholar 

  5. Noda Y, Watanabe H, Lwafuchi M, et al. Carcinoids and endocrine cell micronests of the minor and major duodenal papillae Their incidence and characteristics. Cancer. 1992;70:1825–33.

    Article  CAS  PubMed  Google Scholar 

  6. Binderup T, Knigge U, Loft A, et al. Functional imaging of neuroendocrine tumors: a head-to-head comparison of somatostatin receptor scintigraphy, 123I-MIBG scintigraphy, and 18F-FDG PET. J Nucl Med. 2010;51:704–12.

    Article  PubMed  Google Scholar 

  7. Burke AP, Sobin LH, Federspiel BH, et al. Carcinoid tumors of the duodenum A clinicopathologic study of 99 cases. Arch Pathol Lab Med. 1990;114:700.

    CAS  PubMed  Google Scholar 

  8. Zyromski NJ, Kendrick ML, Nagorney DM, et al. Duodenal carcinoid tumors: How aggressive should we be? J Gastrointest Surg. 2001;5:588–93.

    Article  CAS  PubMed  Google Scholar 

  9. Suda K. Histopathology of the minor duodenal papilla. Dig Surg. 2010;27:137–9.

    Article  PubMed  Google Scholar 

  10. Kamisawa T. Clinical significance of the minor duodenal papilla and accessory pancreatic duct. J Gastroenterol. 2004;39:605–15.

    Article  PubMed  Google Scholar 

  11. Malone MJ, Silverman ML, Braasch JW, et al. Early somatostatinoma of the papilla of the duct of Santorini. Arch Surg. 1985;120:1381–3.

    Article  CAS  PubMed  Google Scholar 

  12. Stömmer PE, Stolte M, Seifert E. Somatostatinoma of vater’s papilla and of the minor papilla. Cancer. 1987;60:232–5.

    Article  PubMed  Google Scholar 

  13. Lowes JR, Rode J, Lees WR, et al. Obstructive pancreatitis: unusual causes of chronic pancreatitis. Br J Surg. 1988;75:1129–33.

    Article  CAS  PubMed  Google Scholar 

  14. Borobia FG, Fabregat J, Jorba R, et al. Exocrine pancreatic insufficiency caused by a somatostatinoma of the minor and major duodenal papilla in a patient with neurofibromatosis. Eur J Surg. 2001;167:154–6.

    Article  CAS  PubMed  Google Scholar 

  15. House MG, Yeo CJ, Schulick RD. Periampullary pancreatic somatostatinoma. Ann Surg Oncol. 2002;9:869–74.

    Article  PubMed  Google Scholar 

  16. Singh VV, Bhutani MS, Draganov P. Carcinoid of the minor papilla in incomplete pancreas divisum presenting as acute relapsing pancreatitis. Pancreas. 2003;27:96–7.

    Article  PubMed  Google Scholar 

  17. Wang HY, Chen MJ, Yang TL, et al. Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera. World J Gastroenterol. 2005;11:3794–6.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Waisberg J, de Matos LL, Waisberg DR, et al. Carcinoid of the minor duodenal papilla associated with pancreas divisum: Case report and review of the literature. Clinics (Sao Paulo). 2006;61:365–8.

    Article  PubMed  Google Scholar 

  19. Itoi T, Sofuni A, Itokawa F, et al. Endoscopic resection of carcinoid of the minor duodenal papilla. World J Gastroenterol. 2007;13:3763–4.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Bettini R, Falconi M, Crippa S, et al. Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen’s disease. World J Gastroenterol. 2007;13:2761–3.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Pedicone R, Adham M, Hervieu V, et al. Long-term survival after pancreaticoduodenectomy for endocrine tumors of the ampulla of Vater and minor papilla. Pancreas. 2009;38:638–43.

    Article  PubMed  Google Scholar 

  22. Kim YG, Kim TN, Kim KO. Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain. BMC Gastroenterol. 2010;10:17.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Nicolas-Perez D, Gimeno-Garcia AZ, Cabrera HA, et al. Carcinoid of the minor duodenal papilla causing upper GI bleeding. Gastrointest Endosc. 2011;74:1146–7.

    Article  PubMed  Google Scholar 

  24. Maruyama T, Shirai Y, Sakata J, et al. A 1.3-cm carcinoid tumor of the minor duodenal papilla with superior mesenteric lymph node metastases. Surgery. 2012;151:340–1.

    Article  PubMed  Google Scholar 

  25. Oller B, Haetta HH, Lorenzo-Zuniga V, et al. Double somatostatinoma and double papillectomy in a patient with type 1 neurofibromatosis (von Recklinghausen’s disease). Endoscopy. 2012;44(2):344–5. https://doi.org/10.1055/s-0032-1309912.

    Article  Google Scholar 

  26. Fukami Y, Kurumiya Y, Mizuno K, et al. A 12-mm carcinoid tumor of the minor duodenal papilla with lymph node metastases. Jpn J Clin Oncol. 2013;43:74–7.

    Article  PubMed  Google Scholar 

  27. Barresi L, Tarantino I, Ligresti D, et al. Endoscopic ultrasound-guided fine-needle aspiration diagnosis of mixed endocrine somatostatinoma of the minor papilla in von Recklinghausen disease. Endoscopy. 2014. https://doi.org/10.1055/s-0034-1390868.

    Article  PubMed  Google Scholar 

  28. Aktas B, Coban S, Simsek Z, et al. Carcinoid tumor of the minor duodenal papilla: a rare entity in elderly individuals. J Am Geriatr Soc. 2014;62:2006–8.

    Article  PubMed  Google Scholar 

  29. Bhandari R, Riddiough G, Lokan J, et al. Somatostatinoma of the minor papilla treated by local excision in a patient with neurofibromatosis type 1. JOP. 2015;16:81–4.

    PubMed  Google Scholar 

  30. Zandomeni MR, Chahdi Beltrame M, de Santibanes M, et al. Major and minor duodenal papilla neuroendocrine tumors in type 1 neurofibromatosis: case report. J Gastrointest Cancer. 2018;49:71–4.

    Article  CAS  PubMed  Google Scholar 

  31. Virgilio E, La Gumina G, Tozzi F, Marrero YC, et al. Neuroendocrine tumor of the minor duodenal papilla an unusual cause of pancreaticoduodenectomy. Am surg. 2016;82:1145–8.

    Article  PubMed  Google Scholar 

  32. Jara Letelier DI, Bonotto ML, Ardengh JC. Somatostatinoma of the minor duodenal papilla associated with pancreas divisum treated by endoscopic papillectomy. Endoscopy. 2016;48(Suppl 1):E135-137.

    PubMed  Google Scholar 

  33. Seo YK, Choi JS. Endoscopic Papillectomy for synchronous major and minor duodenal papilla neuroendocrine tumors. Korean J Gastroenterol. 2018;72:217–21.

    Article  PubMed  Google Scholar 

  34. Vanoli A, Albarello L, Uncini S, et al. Neuroendocrine tumors (nets) of the minor papilla/ampulla: analysis of 16 cases underlines homology with major ampulla NETs and differences from extra-ampullary duodenal NETs. Am J Surg Pathol. 2019;43:725–36.

    Article  PubMed  Google Scholar 

  35. Takagi K, Umeda Y, Yoshida R, et al. Gastroenteropancreatic neuroendocrine tumor of the accessory papilla of the duodenum: a case report. Surg Case Rep. 2021;7:156.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Yamamoto K, Itoi T, Iwasaki E, et al. Endoscopic papillectomy for tumors of the minor duodenal papilla: A case series of six patients and literature review. J Hepatobiliary Pancreat Sci. 2021;21(10):1142–50.

    Article  Google Scholar 

  37. Heo JH, Choi EJ, Yu SJ, et al. Neuroendocrine tumor with metachronous gastrointestinal stromal tumor in a patient: a Case Report. Korean J Gastroenterol. 2022;79:72–6.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Contributions

NA, MW, YT and TI participated in diagnosis and drafted this manuscript. AF, YN and HS supervised this manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Naoki Aoyama.

Ethics declarations

Conflict of interest

All authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Human and animal rights

This article does not contain any studies directly involving human participants. Patient information is fully anonymized.

Inform consent

Informed consent was obtained from the patient for publication of this case report and accompanying images.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Aoyama, N., Wada, M., Taniguchi, Y. et al. A case of neuroendocrine neoplasm of the minor duodenal papilla. Clin J Gastroenterol 16, 171–179 (2023). https://doi.org/10.1007/s12328-022-01739-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12328-022-01739-w

Keywords

Navigation