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Ampulla of Vater Gangliocytic Paraganglioma

MedGen UID:
474118
•Concept ID:
C3272485
•
Neoplastic Process
Synonym: Gangliocytic Paraganglioma of the Ampullary Region

Definition

An extra-adrenal paraganglioma that arises from the ampulla of Vater and the periampullary region. It consists of a mixture of neuroendocrine cells, Schwann-like cells, and ganglion cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpulla of Vater Gangliocytic Paraganglioma

Recent clinical studies

Etiology

Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater.
Sekine M, Miyatani H, Matsumoto K, Kashima H, Koito Y, Miura T, Takahashi Y, Tsuboi R, Ishii T, Fujiwara J, Uehara T, Urayoshi S, Yuhashi K, Asano T, Sagihara N, Matsumoto S, Mashima H
Intern Med 2018 Sep 15;57(18):2663-2668. Epub 2018 May 18 doi: 10.2169/internalmedicine.0464-17. PMID: 29780117Free PMC Article
Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty.
Rafiullah, Tanimu S, Onitilo AA
BMJ Case Rep 2014 Jul 2;2014 doi: 10.1136/bcr-2013-202941. PMID: 25100803Free PMC Article
Tumors of the ampulla of vater: histopathologic classification and predictors of survival.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
J Am Coll Surg 2008 Aug;207(2):210-8. Epub 2008 May 5 doi: 10.1016/j.jamcollsurg.2008.01.028. PMID: 18656049
Periampullary gangliocytic paraganglioma.
Sakhuja P, Malhotra V, Gondal R, Dutt N, Choudhary A
J Clin Gastroenterol 2001 Aug;33(2):154-6. doi: 10.1097/00004836-200108000-00013. PMID: 11468445
Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study.
Altavilla G, Chiarelli S, Fassina A
Ultrastruct Pathol 2001 Mar-Apr;25(2):137-45. doi: 10.1080/019131201750222220. PMID: 11407527

Diagnosis

Ampullary gangliocytic paraganglioma with lymph node metastasis: A case report with literature review.
Choi H, Choi JW, Ryu DH, Park S, Kim MJ, Yoo KC, Woo CG
Medicine (Baltimore) 2022 Apr 15;101(15):e29138. doi: 10.1097/MD.0000000000029138. PMID: 35475800Free PMC Article
Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater.
Sekine M, Miyatani H, Matsumoto K, Kashima H, Koito Y, Miura T, Takahashi Y, Tsuboi R, Ishii T, Fujiwara J, Uehara T, Urayoshi S, Yuhashi K, Asano T, Sagihara N, Matsumoto S, Mashima H
Intern Med 2018 Sep 15;57(18):2663-2668. Epub 2018 May 18 doi: 10.2169/internalmedicine.0464-17. PMID: 29780117Free PMC Article
A case of gangliocytic paraganglioma in the ampulla of Vater.
Kwon J, Lee SE, Kang MJ, Jang JY, Kim SW
World J Surg Oncol 2010 May 24;8:42. doi: 10.1186/1477-7819-8-42. PMID: 20497533Free PMC Article
Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
Arch Surg 2009 Jun;144(6):527-31. doi: 10.1001/archsurg.2009.80. PMID: 19528385
Periampullary gangliocytic paraganglioma.
Sakhuja P, Malhotra V, Gondal R, Dutt N, Choudhary A
J Clin Gastroenterol 2001 Aug;33(2):154-6. doi: 10.1097/00004836-200108000-00013. PMID: 11468445

Therapy

Good outcome from surgery for ampullary tumour.
Toh SK, Davies N, Dolan P, Worthley C, Townsend N, Williams JA
Aust N Z J Surg 1999 Mar;69(3):195-8. doi: 10.1046/j.1440-1622.1999.01521.x. PMID: 10075358

Prognosis

Gangliocytic paraganglioma: an unusual cause of upper gastrointestinal bleeding.
Sánchez Torrijos Y, León Montañés R, Mejías Manzano MÁ
Rev Esp Enferm Dig 2017 Aug;109(8):605-606. doi: 10.17235/reed.2017.4808/2016. PMID: 28715900
Gangliocytic paraganglioma, a rare ampullary tumour treated with open transduodenal resection and sphincteroplasty.
Rafiullah, Tanimu S, Onitilo AA
BMJ Case Rep 2014 Jul 2;2014 doi: 10.1136/bcr-2013-202941. PMID: 25100803Free PMC Article
Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
Arch Surg 2009 Jun;144(6):527-31. doi: 10.1001/archsurg.2009.80. PMID: 19528385
Tumors of the ampulla of vater: histopathologic classification and predictors of survival.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
J Am Coll Surg 2008 Aug;207(2):210-8. Epub 2008 May 5 doi: 10.1016/j.jamcollsurg.2008.01.028. PMID: 18656049
Neuroendocrine tumours of the duodenum. Clinical aspects, pathomorphology and therapy.
Witzigmann H, Loracher C, Geissler F, Wagner T, Tannapfel A, Uhlmann D, Caca K, Hauss J, Hehl JA
Langenbecks Arch Surg 2002 Jan;386(7):525-33. Epub 2001 Nov 6 doi: 10.1007/s00423-001-0260-z. PMID: 11819111

Clinical prediction guides

Gangliocytic Paraganglioma with Carcinoma of the Ampulla of Vater.
Sekine M, Miyatani H, Matsumoto K, Kashima H, Koito Y, Miura T, Takahashi Y, Tsuboi R, Ishii T, Fujiwara J, Uehara T, Urayoshi S, Yuhashi K, Asano T, Sagihara N, Matsumoto S, Mashima H
Intern Med 2018 Sep 15;57(18):2663-2668. Epub 2018 May 18 doi: 10.2169/internalmedicine.0464-17. PMID: 29780117Free PMC Article
Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
Arch Surg 2009 Jun;144(6):527-31. doi: 10.1001/archsurg.2009.80. PMID: 19528385
Tumors of the ampulla of vater: histopathologic classification and predictors of survival.
Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW
J Am Coll Surg 2008 Aug;207(2):210-8. Epub 2008 May 5 doi: 10.1016/j.jamcollsurg.2008.01.028. PMID: 18656049
A metastatic endocrine-neurogenic tumor of the ampulla of Vater with multiple endocrine immunoreaction--malignant paraganglioma?
Büchler M, Malfertheiner P, Baczako K, Krautzberger W, Beger HG
Digestion 1985;31(1):54-9. doi: 10.1159/000199178. PMID: 2858422

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