Skip to main content
Log in

Periampullary Gangliocytic Paraganglioma

  • Original Article
  • Published:
Journal of Gastrointestinal Surgery Aims and scope

Abstract

Background

Gangliocytic paraganglioma (GP) is rare and difficult to be differentiated from other periampullary neoplasms. The clinical characteristics and optimal treatment of periampullary GPs have not been clarified.

Methods

The data pool for the analysis comprised of cases of periampullary GP encountered in our institution and sporadic cases reported in the English literature.

Results

A total of 117 cases with periampullary GP were studied, including 2 from our institute, and among them, duodenal GP was the most common (53.0%). GP size ranged from 0.7 to 19.0 cm, with a median of 2.2 cm. The most common presenting symptom for overall periampullary GPs was epigastric pain in 49.5% cases, followed by gastrointestinal bleeding in 35.4% cases. Most (84.1%) of the periampullary GPs were benign, whereas 15.9% were malignant. Metastasis was noted in 26.3% of periampullary GPs, with 14.5% showing lymph node metastasis and 1.8% showing liver metastasis. Of the periampullary GP cases included, 30.1% were treated with pancreaticoduodenectomy, 40.6% with local excision, and 17.7% with endoscopic resection.

Conclusions

Periampullary GP should be considered as a tumor with malignant potential. Endoscopic resection is the treatment of choice for most of the duodenal GPs, whereas pancreaticoduodenectomy is recommended for GPs with possible malignancy, such as large size, with submucosal invasion, or pancreatic GP.

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

Similar content being viewed by others

References

  1. Okubo Y, Wakayama M, Nemoto T, Kitahara K, Nakayama H, Shibuya K, Yokose T, Yamada M, Shimodaira K, Sasai D, Ishiwatari T, Tsuchiya M, Hiruta N. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187–194.

    PubMed  PubMed Central  Google Scholar 

  2. Okubo Y, Yokose T, Motohashi O, Miyagi Y, Yoshioka E, Suzuki M, Washimi K, Kawachi K, Nito M, Nemoto T, Shibuya K, Kameda Y. Duodenal Rare Neuroendocrine Tumor: Clinicopathological Characteristics of Patients with Gangliocytic Paraganglioma. Gastroenterol Res Pract 2016;2016:5257312.

    PubMed  PubMed Central  Google Scholar 

  3. Hernandez AG, Lanuza ED, Matias AC, Huertas RP, Rodriguez KM, Perez PG, Mompean FO: Large gangliocytic paraganglioma of the duodenum: A rare entity. World J Gastrointest Surg 2015;7(8):170–173.

    PubMed  PubMed Central  Google Scholar 

  4. Tatangelo F, Cantile M, Pelella A, Losito NS, Scognamiglio G, Bianco F, Belli A, Botti G. Duodenal gangliocytic paraganglioma, a rare entity among GEP-NET. a case report with immunohistochemical and molecular study. Diagn Pathol 2014;9:54–58.

    PubMed  PubMed Central  Google Scholar 

  5. Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas; brief review with report of a duodenal ganglioneuroma. The American journal of pathology 1957;33(5):953–965.

    CAS  PubMed  PubMed Central  Google Scholar 

  6. Kepes JJ, Zacharias DL. Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination. Cancer 1971;27(1):61–67.

    CAS  PubMed  Google Scholar 

  7. Okubo Y, Nemoto T, Wakayama M, Tochigi N, Shinozaki M, Ishiwatari T, Aki K, Tsuchiya M, Aoyama H, Katsura K, Fujii T, Nishigami T, Yokose T, Ohkura Y, Shibuya K. Gangliocytic paraganglioma: a multi-institutional retrospective study in Japan. BMC Cancer 2015;15:269–277.

    PubMed  PubMed Central  Google Scholar 

  8. Papaconstantinou D, Machairas N, Damaskou V, Zavras N, Kontopoulou C, Machairas A. Duodenal gangliocytic paraganglioma, successfully treated by local surgical excision-a case report. Int J Surg Case Rep 2017;32:5–8.

    PubMed  PubMed Central  Google Scholar 

  9. Paasch C, Hunerbein M, Theissig F. Endoscopic resection of a periampullary gangliocytic paraganglioma of the duodenum. A case report. Int J Surg Case Rep 2016;29:39–43.

    PubMed  PubMed Central  Google Scholar 

  10. Colle E, Zouhry I, Colignon N, Mourra N. Paraampullary gangliocytic paraganglioma. Clin Res Hepatol Gastroenterol 2017;42(4):291–293.

    PubMed  Google Scholar 

  11. Cathcart SJ SA, Kozel JA, Oliveto JM, Ly QP. Duodenal gangliocytic paraganglioma with lymph node metastases A case report and comparative review of 31 cases. World J Clin Cases 2017;5(6):222–233.

    PubMed  PubMed Central  Google Scholar 

  12. Matsubayashi H, Ishiwatari H, Matsui T, Fujie S, Uesaka K, Sugiura T, Okamura Y, Yamamoto Y, Ashida R, Ito T, Sasaki K, Ono H. Gangliocytic Paraganglioma of the Minor Papilla of the Duodenum. Intern Med 2017;56(9):1029–1035.

    PubMed  PubMed Central  Google Scholar 

  13. Nonaka K, Matsuda Y, Okaniwa A, Kasajima A, Sasano H, Arai T. Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review. Diagn Pathol 2017;12(1):57–63.

    PubMed  PubMed Central  Google Scholar 

  14. Park HK HH. Duodenal Gangliocytic Paraganglioma With Lymph Node Metastasis. Arch Pathol Lab Med 2016;140(1):94–98.

    CAS  PubMed  Google Scholar 

  15. Guerges M, Slama E, Maskoni B, Imlay S, McKany M. Gangliocytic paraganglioma treated with ampullectomy, A case report. Ann Med Surg (Lond) 2016;11:16–20.

    Google Scholar 

  16. Ginesu GC, Barmina M, Paliogiannis P, Trombetta M, Cossu ML, Feo CF, Addis F, Porcu A. Nonfunctional paraganglioma of the head of the pancreas: A rare case report. Int J Surg Case Rep 2016;28:81–84.

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Meng L, Wang J, Fang SH. Primary pancreatic paraganglioma: a report of two cases and literature review. World J Gastroenterol 2015;21(3):1036–1039.

    PubMed  PubMed Central  Google Scholar 

  18. Loftus TJ, Kresak JL, Gonzalo DH, Sarosi GA, Jr., Behrns KE. Duodenal gangliocytic paraganglioma: A case report and literature review. Int J Surg Case Rep 2015;8C:5–8.

    PubMed  Google Scholar 

  19. Dowden JE S-OCK, Kimchi ET, Camp ER, Morgan KA, Adams DB. Ampullary Gangliocytic Paraganglioma with Lymph Node Metastasis. Am Surg 2015;81(11):E359–360.

    Google Scholar 

  20. Boeriu A, Dobru D, Georgescu R, Mocan S, Boeriu C. Gangliocytic paraganglioma: a rare cause of gastrointestinal bleeding. J Gastrointestin Liver Dis 2015;24(1):109–112.

    PubMed  Google Scholar 

  21. Zhang L LQ, Hu Y, Zhao Y. Paraganglioma of the pancreas a potentially functional and malignant tumor. World J Surg Oncol 2014;12:218–220.

    PubMed  PubMed Central  Google Scholar 

  22. Yang JI, Choi JS, Lee GH, Kim BW, Moon SJ, Kang MS, Ahn HJ. A case of ampullary gangliocytic paraganglioma. Korean J Intern Med 2014;29(3):375–378.

    PubMed  PubMed Central  Google Scholar 

  23. Rafiullah, Tanimu S, Onitilo AA. Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty. BMJ Case Rep 2014;pii: bcr2013202941. https://doi.org/10.1136/bcr-2013-202941.

    Google Scholar 

  24. Park SJ, Kim DH, Lim H, Lee JH, Choi KD, Song HJ, Lee GH, Jung H-Y, Kim J-H, Park JY. Endoscopic Resection as a Possible Radical Treatment for Duodenal Gangliocytic Paraganglioma: A Report of Four Cases. The Korean Journal of Gastroenterology 2014;63(2):114–119.

    PubMed  Google Scholar 

  25. Li B LY, Tian XY, Luo BN, Li Z. Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course. World J Gastroenterol 2014;20(41):15454–15461.

    PubMed  PubMed Central  Google Scholar 

  26. Feuerstein JD, Yee EU, Pleskow D. Not every ampullary mass is deadly. Gastroenterology 2014;147(6):e3–4.

    PubMed  Google Scholar 

  27. Asimakopoulou NI PP, Lasithiotakis KG, Mouzas IA, Daskalogiannaki ME, Tzardi MN, Chalkiadakis GE. Recurrent episodes of acute pancreatitis due to duodenal gangliocytic paraganglioma report of a case. JOP 2014;15(2):201–205.

    PubMed  Google Scholar 

  28. Sathyamurthy A, Choudhary A, Ng D, Okponobi S, Diaz-Arias A, Grewal A, Hammoud GM. Obstructive jaundice due to a rare periampullary tumor. World J Gastrointest Oncol 2013;5(10):195–197.

    PubMed  PubMed Central  Google Scholar 

  29. Prakash K KN, Mathew P. Malignant paraganglioma of the bile duct. Trop Gastroenterol 2013;34(3):180–182.

    PubMed  Google Scholar 

  30. Narang V, Behl N, Sood N, Puri H. Gangliocytic paraganglioma of duodenum. Case Rep Pathol 2013;2013:378582.

    PubMed  PubMed Central  Google Scholar 

  31. Dustin SM, Atkins KA, Shami VM, Adams RB, Stelow EB. The cytologic diagnosis of gangliocytic paraganglioma: a case report. Diagn Cytopathol 2013;41(7):650–653.

    PubMed  Google Scholar 

  32. Al-Jiffry BO AY, Khayat SH, Haiba M, Hatem M. Malignant extra-adrenal pancreatic paraganglioma case report and literature review. BMC Cancer 2013;13:486–490.

    PubMed  PubMed Central  Google Scholar 

  33. Wu GC WK, Zhang ZT. Gangliocytic paraganglioma of the duodenum a case report. Chin Med J (Engl) 2012;125(2):388–389.

    Google Scholar 

  34. Nuno-Guzman CM, Arroniz-Jauregui J, Alvarez-Lopez F, Corona JL, Cerda-Camacho F, Rostro R, Gutierrez-Manjarrez JI. Obstructing gangliocytic paraganglioma in the third portion of the duodenum. Case Rep Gastroenterol 2012;6(2):489–495.

    PubMed  PubMed Central  Google Scholar 

  35. Higa B KU. Malignant paraganglioma of the pancreas. Pathology 2012;44(1):53–55.

    PubMed  Google Scholar 

  36. Čečka F JB, Repák R, Kohout A, Šubrt Z, Ferko A. Gangliocytic paraganglioma of the duodenum. Can J Gastroenterol 2012;26(11):778–779.

    PubMed  PubMed Central  Google Scholar 

  37. Barret M, Rahmi G, Duong van Huyen JP, Landi B, Cellier C, Berger A. Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report. Eur J Gastroenterol Hepatol 2012;24(1):90–94.

    PubMed  Google Scholar 

  38. Assef MS, Carbonari AP, Araki O, Nakao F, Marchetti I, Medeiros MT, Kassab P, Malheiros CA, Rossini LB. Gangliocytic paraganglioma of the duodenal papilla associated with esophagogastric adenocarcinoma. Endoscopy 2012;44 Suppl 2 UCTN:E165–166.

    PubMed  Google Scholar 

  39. Parray FQ, Lone IM, Chowdri NA, Wani I, Wani MA, Gulzar GM, Thakur N. Emergency pancreaticoduodenectomy in duodenal paraganglioma: case report. ISRN Surg 2011:268674.

  40. Lytras D KE, Hatfield AR, Vyas S, Imber CJ, Webster GJ, Malagó M. Recurrent acute pancreatitis as primary manifestation of gangliocytic paraganglioma of the ampulla. pancreas 2011;40(6):985–986.

    PubMed  Google Scholar 

  41. Lightfoot N SP, Nikfarjam M. Paraganglioma mimicking a pancreatic neoplasm. JOP 2011;12(3):259–261.

    PubMed  Google Scholar 

  42. He J, Zhao F, Li H, Zhou K, Zhu B. Pancreatic paraganglioma: A case report of CT manifestations and literature review. Quant Imaging Med Surg 2011;1(1):41–43.

    PubMed  PubMed Central  Google Scholar 

  43. Von Loga K KA, Zustin J. Periampullary gangliocytic paraganglioma a case report. In Vivo 2010;24(3):321–324.

    Google Scholar 

  44. Sucandy I AG, Bertsch DJ. Surgical management of endoscopically unresectable duodenal gangliocytic paraganglioma in a patient with partial upper gastrointestinal obstruction. N Am J Med Sci 2010;2(11):547–551.

    PubMed  PubMed Central  Google Scholar 

  45. Okubo Y YT, Tuchiya M, Mituda A, Wakayama M, Hasegawa C, Sasai D, Nemoto T, Shibuya K. Duodenal gangliocytic paraganglioma showing lymph node metastasis a rare case report. Diagn Pathol 2010;5:27.

    PubMed  PubMed Central  Google Scholar 

  46. Kwon J LS, Kang MJ, Jang JY, Kim SW. A case of gangliocytic paraganglioma in the ampulla of Vater. World J Surg Oncol 2010;24(8):42.

    Google Scholar 

  47. Ahn KS HH, Yoon YS, Cho JY, Khalikulov K. Laparoscopic transduodenal ampullectomy for benign ampullary tumors. J Laparoendosc Adv Surg Tech A 2010;20(1):59–63.

    PubMed  Google Scholar 

  48. Ohtsuki Y, Watanabe R, Kimura M, Okamoto T, Murakami S, Mizukami Y, Takeji M, Okada Y, Hayashi Y, Lee GH, Furihata M. Immunohistochemical and electron microscopic studies of a case of duodenal gangliocytic paraganglioma. Med Mol Morphol 2009;42(4):245–249.

    PubMed  Google Scholar 

  49. Ghassemi KA, Cortina G, Reber HA, Farrell JJ. Complete Resection of Ampullary Paragangliomas Confined to the Submucosa on Endoscopic Ultrasound May Be Best Achieved by Radical Surgical Resection. Case Rep Gastroenterol 2009;3(2):169–174.

    PubMed  PubMed Central  Google Scholar 

  50. Chen J SE, Morgenstern S, Geller A, Greif F. Local surgical resection of gangliocytic paraganglioma of the duodenal papilla. Isr Med Assoc J 2009;11(5):311–312.

    PubMed  Google Scholar 

  51. Kim SY, Byun JH, Choi G, Yu E, Choi EK, Park SH, Lee MG. A case of primary paraganglioma that arose in the pancreas: the Color Doppler ultrasonography and dynamic CT features. Korean J Radiol 2008;9 Suppl:S18–21.

    PubMed  PubMed Central  Google Scholar 

  52. Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg 2007;11(10):1351–1354.

    PubMed  Google Scholar 

  53. Morita T, Tamura S, Yokoyama Y, Onishi T, Kuratani Y, Mizuta H, Onishi S. Endoscopic resection of a duodenal gangliocytic paraganglioma. Dig Dis Sci 2007;52(6):1400–1404.

    PubMed  Google Scholar 

  54. Plaza JA, Vitellas K, Marsh WL. Duodenal gangliocytic paraganglioma: a radiological-pathological correlation. Annals of Diagnostic Pathology 2005;9(3):143–147.

    PubMed  Google Scholar 

  55. Ohkawara T NH, Takeda H, Asaka M. Primary paraganglioma of the head of pancreas contribution of combinatorial image analyses to the diagnosis of disease. Intern Med 2005;44(11):1195–1196.

    PubMed  Google Scholar 

  56. El Idrissi-Lamghari A, Rioux-Leclercq N, Pagenault M, Bretagne JF: Voluminous juxtapapillary gangliocytic paraganglioma. Gastrointest Endosc 2005;62(3):445–446.

    PubMed  Google Scholar 

  57. Sánchez-Pobre P S-LS, Rodríguez S, Sánchez F, Alemany I, López G, Colina F, Martínez-Montiel P, Marín JC, Castellano G, Solís Herruzo JA. Safe endoscopic resection of gangliocytic paraganglioma of the major duodenal papilla. Rev Esp Enferm Dig 2004;96(9):660–662.

    PubMed  Google Scholar 

  58. Nagai T, Torishima R, Nakashima H, Tanahashi J, Iwata M, Ookawara H, Yokoyama S, Yada K, Sato R, Murakami K, Fujioka T. Duodenal gangliocytic paraganglioma treated with endoscopic hemostasis and resection. J Gastroenterol 2004;39(3):277–283.

    PubMed  Google Scholar 

  59. Bucher P MZ, Bühler L, Chilcott M, Gervaz P, Egger JF, Morel P. Paraganglioma of the ampulla of vater a potentially malignant neoplasm. Scand J Gastroenterol 2004;39(3):291–295.

    CAS  PubMed  Google Scholar 

  60. Sundararajan V R-ST, Lowy AM. Duodenal gangliocytic paraganglioma with lymph node metastasis a case report and review of the literature. Arch Pathol Lab Med 2003;127(3):e139–141.

    PubMed  Google Scholar 

  61. Nakamura T, Ozawa T, Kitagawa M, Takehira Y, Yamada M, Yasumi K, Tamakoshi K, Kobayashi Y, Nakamura H. Endoscopic resection of gangliocytic paraganglioma of the minor duodenal papilla: case report and review. Gastrointest Endosc 2002;55(2):270–273.

    PubMed  Google Scholar 

  62. Girgis PA HW. Pathologic quiz case. Mass at the ampulla of vater in a 43-year-old man. Gangliocytic paraganglioma. Arch Pathol Lab Med 2002;126(10):1239–1240.

    PubMed  Google Scholar 

  63. Sakhuja P MV, Gondal R, Dutt N, Choudhary A. Periampullary gangliocytic paraganglioma. J Clin Gastroenterol 2001;33(2):154–156.

    CAS  PubMed  Google Scholar 

  64. Altavilla G CS, Fassina A. Duodenal periampullary gangliocytic paraganglioma report of two cases with immunohistochemical and ultrastructural study. Ultrastruct Pathol 2001;25(2):137–145.

    CAS  PubMed  Google Scholar 

  65. Parithivel VS NM, Malhotra AK, Swaminathan K, Kaul A, Shah AK. Paraganglioma of the pancreas literature review and case report. Dig Dis Sci 2000;45(2):438–441.

    CAS  PubMed  Google Scholar 

  66. Kotsis T VD, Paphiti A, Frangou M, Mallas E. Duodenal gangliocytic paraganglioma as a radiological moving defect. dig Surg 2000;17(6):636–640.

    CAS  PubMed  Google Scholar 

  67. Kehagias DT ML, Pafiti A, Vlahos LJ. Quiz case of the month. Diagnosis duodenal gangliocytic paraganglioma. Eur Radiol 1999;9(7):1465–1466.

    CAS  PubMed  Google Scholar 

  68. Kehagias DT ML, Pafiti A, Vlahos LJ. Gangliocytic paraganglionoma of the duodenum. Eur Radiol 1999;9(7):1465–1466.

    CAS  PubMed  Google Scholar 

  69. Fujino Y NY, Ogino K, Watahiki H, Ogawa H, Saitoh Y. Nonfunctional paraganglioma of the pancreas report of a case. Surg Today 1998;28(2):209–212.

    CAS  PubMed  Google Scholar 

  70. Reed RJ CPJ, Harkin JC. Gangliocytic paraganglioma. Am J Surg Pathol 1997;1(3):207–216.

    Google Scholar 

  71. Buetow PC LM, Buck JL, Pantongrag-Brown L, Emory TS. Duodenal gangliocytic paraganglioma CT, MR imaging, and US findings. Radiology 1997;204(3):745–747.

    CAS  PubMed  Google Scholar 

  72. Evans JD WP, Barber PC, Neoptolemos JP. Duodenal gangliocytic paraganglioma presenting as an ampullary tumor. Int J Pancreatol 1996;20(2):131–134.

    CAS  PubMed  Google Scholar 

  73. Hashimoto S KS, Matsuzawa K, Harada H, Makuuchi M. Gangliocytic paraganglioma of the papilla of Vater with regional lymph node metastasis. Am J Gastroenterol 1992;87(9):1216–1218.

    CAS  PubMed  Google Scholar 

  74. Imai S KY, Komaki K, Nishishita S, Fukuya T. Paraganglioma of the duodenum a case report with radiological findings and literature review. Br J Radiol 1990;63(756):975–977.

    CAS  PubMed  Google Scholar 

  75. Perrone T. Duodenal gangliocytic paraganglioma and carcinoid. Am J Surg Pathol 1986;10(2):147–149.

    CAS  PubMed  Google Scholar 

  76. Scheithauer BW NF, LeChago J, Wick MR, Crawford BG, Weiland LH, Carney JA. Duodenal gangliocytic paraganglioma. Clinicopathologic and immunocytochemical study of 11 cases. Am J Clin Pathol 1986;86(5):559–565.

    CAS  PubMed  Google Scholar 

  77. Sando WC MS, Rodgers BM. Duodenal gangliocytic paraganglioma occurring in adolescence. J Pediatr Gastroenterol Nutr 1986;5(4):659–664.

    CAS  PubMed  Google Scholar 

  78. Perrone T SR, Rosai J. Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. Am J Surg Pathol 1985;9(1):31–41.

    CAS  PubMed  Google Scholar 

  79. Büchler M MP, Baczako K, Krautzberger W, Beger HG. A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction--malignant paraganglioma. Digestion 1985;31(1):54–59.

    PubMed  Google Scholar 

  80. Guarda LA ON, del Junco GW, Luna MA. Gangliocytic paraganglioma of the duodenum an immunocytochemical study. Am J Gastroenterol 1983;78(12):794–798.

    CAS  PubMed  Google Scholar 

  81. Cohen T ZS, Tallis A, Tuazon R, Reich M.Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship. Am J Gastroenterol 1981;75(3):197–203.

    CAS  Google Scholar 

  82. Beltrami CA MR, Cinti S. Paraganglioma of the duodenum a case report. Tumori 1980;66(5):637–641.

    CAS  PubMed  Google Scholar 

  83. Meneghetti AT, Safadi B, Stewart L, Way LW. Local resection of ampullary tumors. J Gastrointest Surg 2005;9(9):1300–1306.

    PubMed  Google Scholar 

Download references

Financial Support

This work was financially supported by grants from Taipei Veterans General Hospital (V106C-028, VTA106-V1-1-1) and the Ministry of Science and Technology (MOST 106-2314-B-075 -048 -MY2) and the Ministry of Health and Welfare (MOHW107-TDU-B-212-114026A).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Shin-E Wang.

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

Chiang, CS., Shyr, BU., Chen, SC. et al. Periampullary Gangliocytic Paraganglioma. J Gastrointest Surg 23, 2247–2254 (2019). https://doi.org/10.1007/s11605-018-4019-z

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11605-018-4019-z

Keywords

Navigation