Skip to main content
Log in

Surgical treatment for a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis

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

Abstract

We report a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. An asymptomatic 58-year-old woman was admitted for the evaluation of abnormal liver enzyme test results. Abdominal computed tomography and upper gastrointestinal endoscopy revealed a 27-mm pedunculated tumor of the papilla of Vater. Adenocarcinoma of the papilla with muscularis propria invasion was suspected; therefore, pancreaticoduodenectomy with lymph node dissection was performed. There were no perioperative complications. A definitive diagnosis of gangliocytic paraganglioma of the papilla of Vater with lymph node metastases was confirmed by pathological examinations, which is a rare condition. The patient was discharged on her 16th postoperative day, and no recurrence or metastases were identified during the 18-month follow-up period. We herein discuss the surgical treatment for this rare case and present a review of related literature.

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

Similar content being viewed by others

Abbreviations

GP:

Gangliocytic paraganglioma

CEA:

Carcinoembryonic antigen

CA19-9:

Carbohydrate antigen 19-9

CT:

Computed tomography

LI:

Labeling index

LNM:

Lymph node metastasis

References

  1. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer. 2011;11:187.

    Article  Google Scholar 

  2. Rowsell C, Coburn N, Chetty R. Gangliocytic paraganglioma: a rare case with metastases of all 3 elements to liver and lymph nodes. Ann Diagn Pathol. 2011;15:467–71.

    Article  Google Scholar 

  3. Amin SM, Albrechtsen NW, Forster J, et al. Gangliocytic paraganglioma of duodenum metastatic to lymph nodes and liver and extending into the retropancreatic space. Pathologica. 2013;105:90–3.

    CAS  PubMed  Google Scholar 

  4. Li B, Li Y, Tian XY, et al. Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course. World J Gastroenterol. 2014;20:15454–61.

    Article  Google Scholar 

  5. Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas; brief review with report of a duodenal ganglioneuroma. Am J Pathol. 1957;33:953–65.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Okubo Y, Yokose T, Motohashi O, et al. Duodenal rare neuroendocrine tumor: clinicopathological characteristics of patients with gangliocytic paraganglioma. Gastroenterol Res Pract. 2016;2016:5257312.

    Article  Google Scholar 

  7. Burke AP, Helwig EB. Gangliocytic paraganglioma. Am J Clin Pathol. 1989;92:1–9.

    Article  CAS  Google Scholar 

  8. Weinrach DM, Wang KL, Blum MG, et al. Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus. Hum Pathol. 2004;35:1288–91.

    Article  Google Scholar 

  9. Henry C, Ghalel-Mechaoui H, Bottero N, et al. Gangliocytic paraganglioma of the pancreas with bone metastasis. Ann Chir. 2003;128:336–8.

    Article  CAS  Google Scholar 

  10. Van ES, Offerhaus GJ, Peterse HL, et al. Gangliocytic paraganglioma of the appendix. Histopathology. 1993;63:2540–5.

    Google Scholar 

  11. Hironaka M, Fukayama M, Takayashiki N, et al. Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms. Am J Surg Pathol. 2001;25:688–93.

    Article  CAS  Google Scholar 

  12. Ebi M, Ochiai T, Sugiyama T, et al. Endoscopic resection of gangliocytic paraganglioma of the duodenum: a case report. Clin J Gastroenterol. 2020;13(2):203–8.

    Article  Google Scholar 

  13. Sugiura K, Suzuki D, Akiyama T, et al. A case of gangliocytic paraganglioma of the papilla of vater. J Jpn Surg Assoc. 2012;73:346–52.

    Article  Google Scholar 

  14. Sahara JC, Aaron RS, Jessica AK, et al. Duodenal gangliocytic paraganglioma with lymph node metastases: a case report and comperative review of 31 cases. World J Clin Cases. 2017;5:222–33.

    Article  Google Scholar 

  15. Jorge A-S, Donald EH, David SK. Atlas of tumor pathology: tumors of the gallbladder, extrahepatic bile ducts, and vaterian system. American Registry of Pathology; 2016. p. 547–56.

  16. Buchler M, Malfertheiner P, Baczako K, et al. A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction—malignant paraganglioma? Digestion. 1985;31:54–9.

    Article  CAS  Google Scholar 

  17. Okubo Y, Yoshioka E, Suzuki M, et al. Diagnosis, pathological findings, and clinical management of gangliocytic paraganglioma: a systematic review. Front Oncol. 2018;8:291.

    Article  Google Scholar 

Download references

Acknowledgements

This work was supported by Grants-in-Aid for Research from the National Center for Global Health and Medicine (30-1021to N.T.).

Funding

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Nobuyuki Takemura.

Ethics declarations

Conflict of interest

The authors disclose no conflicts.

Additional information

Publisher's Note

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

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Miyazaki, T., Takemura, N., Mihara, F. et al. Surgical treatment for a rare case of duodenal gangliocytic paraganglioma accompanied with lymph node metastasis. Clin J Gastroenterol 15, 712–716 (2022). https://doi.org/10.1007/s12328-022-01636-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12328-022-01636-2

Keywords

Navigation