Skip to main content
Log in

Rare case of a gallbladder neuroendocrine carcinoma

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

Abstract

There are no neuroectodermal cells in the gallbladder mucosa. Therefore, gallbladder neuroendocrine carcinoma (NEC) is extremely rare and has a poor prognosis. We report a case of a Japanese man in his 60s with this disease. The patient visited a family doctor for epigastralgia. Blood tests showed no abnormalities, including tumor markers, such as CEA and CA19-9. Abdominal ultrasonography (US) showed a low-echoic mass, 39 × 30 mm, with clear boundaries to the liver from the fundus of the gallbladder. Contrast-enhanced computed tomography showed that the tumor was enhanced early and washed out. Diffusion-weighted MRI showed a high signal. We suspected liver invasion of gallbladder cancer and performed a cholecystectomy, S4 and S5 hepatectomy, and lymphadenectomy. The resected whitish tumor was 29 × 22 mm. The tumor cells had honeycomb growth to the liver from the gallbladder. Tumor cells were poorly differentiated, and there was no stricture of the gland duct. Immunostaining showed that the tumor cells were positive for CD56, chromogranin A and synaptophysin, and about 30% were positive for Ki-67. Our diagnosis was gallbladder NEC with liver invasion. Although most malignant gallbladder tumors are adenocarcinomas, this case indicates that gallbladder NEC should be considered as a differential diagnosis of gallbladder tumor.

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

Similar content being viewed by others

References

  1. Rothenstein J, Cleary SP, Pond GR, et al. Neuroendocrine tumors of the gastrointestinal tract: a decade of experience at the Princess Margaret Hospital. Am J Clin Oncol. 2008;31:64–70.

    Article  PubMed  Google Scholar 

  2. Oberg K. Diagnostic work-up of gastroenteropancreatic neuroendocrine tumors. Clinics (Sao Paulo). 2012;67:109–12.

    Article  Google Scholar 

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

    Article  PubMed  Google Scholar 

  4. Modlin IM, Shapiro MD, Kidd M. An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg. 2005;29:92–101.

    Article  PubMed  Google Scholar 

  5. Yao JC, Hassan M, Phan A, et al. One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063–72.

    Article  PubMed  Google Scholar 

  6. Chen C, Wang L, Liu X, et al. Gallbladder neuroendocrine carcinoma: report of 10 cases and comparision of clinicopathologic features with gallbladder adenocarcinoma. Int J Clin Exp Pathol. 2015;8:8218–8226

    PubMed  PubMed Central  Google Scholar 

  7. Hauso O, Gustafsson BI, Kidd M, et al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer. 2008;113:2655–64.

    Article  PubMed  Google Scholar 

  8. Chen H, Shen YY, Ni XZ. Two cases of neuroendocrine carcinoma of the gallbladder. World J Gastroenterol. 2014;20:11916–20.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Amin MB, Greene FL, Edge SB, et al. The Eighth Edition AJCC Cancer Staging Manual: Continuing to build a bridge from a population-based to a more “personalized” approach to cancer staging. CA Cancer J Clin. 2017;67:93–9.

    Article  PubMed  Google Scholar 

  10. Monier A, Saloum N, Szmigielski W, et al. Neuroendocrine tumor of the gallbladder. Pol J Radiol. 2015;80:228–31.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Hu HJ, Zhou RX, Tan YQ, et al. Coexisting cancers: a mixture of neuroendocrine carcinoma and adenocarcinoma in the gallbladder: A case report. Medicine (Baltimore). 2016;95:5281.

    Article  Google Scholar 

  12. Albores-Saavedra J. Carcinoid tumors and small-cell carcinomas of the gallbladder and extrahepatic bile ducts: a comparative study based on 221 cases from the Surveillance, Epidemiology, and End Results Program. Ann Diagn Pathol. 2009;13:378–83.

    Article  PubMed  Google Scholar 

  13. Nemenqani DM, Fuloria J, Karam RA, et al. Gallbladder Neuroendocrine Neoplasms: A Case Report of Gallbladder Small Cell Carcinoma. J Gastrointest Cancer. 2016;47:432–5.

    Article  PubMed  Google Scholar 

  14. Maitra A, Tascilar M, Hruban RH, et al. Small cell carcinoma of the gallbladder: a clinicopathologic, immunohistochemical, and molecular pathology study of 12 cases. Am J Surg Pathol. 2001;25:595–601.

    Article  CAS  PubMed  Google Scholar 

  15. Adachi T, Haraguchi M, Irie J, et al. Gallbladder small cell carcinoma: a case report and literature review. Surg Case Rep. 2016;2:71.

    Article  PubMed  PubMed Central  Google Scholar 

  16. 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 

  17. Garin E, Le Jeune F, Devillers A, et al. Predictive value of 18F-FDG PET and somatostatin receptor scintigraphy in patients with metastatic endocrine tumors. J Nucl Med. 2009;50:858–64.

    Article  CAS  PubMed  Google Scholar 

  18. Kubota K, Okasaki M, Minamimoto R, et al. Lesion-based analysis of (18)F-FDG uptake and (111)In-Pentetreotide uptake by neuroendocrine tumors. Ann Nucl Med. 2014;28:1004–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Hashim YM, Trinkaus KM, Linehan DC, et al. Regional lymphadenectomy is indicated in the surgical treatment of pancreatic neuroendocrine tumors (PNETs). Ann Surg. 2014;259:197–203.

    Article  PubMed  Google Scholar 

  20. Papotti M, Cassoni P, Sapino A, et al. Large cell neuroendocrine carcinoma of the gallbladder: report of two cases. Am J Surg Pathol. 2000;24:1424–8.

    Article  CAS  PubMed  Google Scholar 

  21. Jun SR, Lee JM, Han JK, et al. High-grade neuroendocrine carcinomas of the gallbladder and bile duct: Report of four cases with pathological correlation. J Comput Assist Tomogr. 2006;30:604–9.

    Article  PubMed  Google Scholar 

  22. Noske A, Pahl S. Combined adenosquamous and large-cell neuroendocrine carcinoma of the gallbladder. Virchows Arch. 2006;449:135–6.

    Article  PubMed  Google Scholar 

  23. Oshiro H, Matsuo K, Mawatari H, et al. Mucin-producing gallbladder adenocarcinoma with focal small cell and large cell neuroendocrine differentiation associated with pancreaticobiliary maljunction. Pathol Int. 2008;58:780–6.

    Article  PubMed  Google Scholar 

  24. Shimono C, Suwa K, Sato M, et al. Large cell neuroendocrine carcinoma of the gallbladder: long survival achieved by multimodal treatment. Int J Clin Oncol. 2009;14:351–5.

    Article  PubMed  Google Scholar 

  25. Iype S, Mirza TA, Propper DJ, et al. Neuroendocrine tumours of the gallbladder: three cases and a review of the literature. Postgrad Med J. 2009;85:213–8.

    Article  CAS  PubMed  Google Scholar 

  26. Lin D, Suwantarat N, Kwee S, et al. Cushing’s syndrome caused by an ACTH-producing large cell neuroendocrine carcinoma of the gallbladder. World J Gastrointest Oncol. 2010;2:56–8.

    Article  PubMed  PubMed Central  Google Scholar 

  27. Sato K, Imai T, Shirota Y, et al. Combined large cell neuroendocrine carcinoma and adenocarcinoma of the gallbladder. Pathol Res Pract. 2010;206:397–400.

    Article  PubMed  Google Scholar 

  28. Paniz Mondolfi AE, Slova D, Fan W, et al. Mixed adenoneuroendocrine carcinoma (MANEC) of the gallbladder: a possible stem cell tumor? Pathol Int. 2011;61:608–14.

    Article  PubMed  Google Scholar 

  29. Al-Brahim N, Albannai R. Combined large cell neuroendocrine carcinoma and adenocarcinoma of the gallbladder. Endocr Pathol. 2013;24:110–3.

    Article  PubMed  Google Scholar 

  30. Okuyama Y, Fukui A, Enoki Y, et al. A large cell neuroendocrine carcinoma of the gall bladder: diagnosis with 18FDG-PET/CT-guided biliary cytology and treatment with combined chemotherapy achieved a long-term stable condition. Jpn J Clin Oncol. 2013;43:571–4.

    Article  PubMed  Google Scholar 

  31. Nakagawa T, Sakashita N, Ohnishi K, et al. Imprint cytological feature of large cell neuroendocrine carcinoma of the gallbladder: a case report. J Med Invest. 2013;60:149–53.

    Article  PubMed  Google Scholar 

  32. Meguro Y, Fukushima N, Koizumi M, et al. A case of mixed adenoneuroendocrine carcinoma of the gallbladder arising from an intracystic papillary neoplasm associated with pancreaticobiliary maljunction. Pathol Int. 2014;64:465–71.

    Article  PubMed  Google Scholar 

  33. Russo S, Russo F, Maiello FM, et al. Biphasic large cell neuroendocrine carcinoma–pure mucinous carcinoma of the gallbladder (MANEC): a unique combination. Pathologica. 2012;104:185–9.

    CAS  PubMed  Google Scholar 

  34. Liu W, Wang L, He XD, et al. Mixed large cell neuroendocrine carcinoma and adenocarcinoma of the gallbladder: a case report and brief review of the literature. World J Surg Oncol. 2015;13:114.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Tang LH, Basturk O, Sue JJ, et al. A practical approach to the classification of WHO grade 3 (G3) well-differentiated neuroendocrine tumor (WD-NET) and poorly differentiated neuroendocrine carcinoma (PD-NEC) of the pancreas. Am J Surg Pathol. 2016;40:1192–202.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Funding

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Masakuni Fujii.

Ethics declarations

Conflict of interest

We do not have any financial relationships with a biotechnology manufacturer, a pharmaceutical company, or other commercial entity that has any interest in the subject matter connected with our case report.

Human rights

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

Informed consent

The patient gave his informed consent to be included in this report.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Fujii, M., Saito, H. & Shiode, J. Rare case of a gallbladder neuroendocrine carcinoma. Clin J Gastroenterol 12, 38–45 (2019). https://doi.org/10.1007/s12328-018-0883-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12328-018-0883-z

Keywords

Navigation