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Ampulla of Vater Neuroendocrine Carcinoma

MedGen UID:
474113
Concept ID:
C3272480
Neoplastic Process
Synonyms: Ampulla of Vater High Grade Neuroendocrine Carcinoma; Ampulla of Vater NEC; Ampulla of Vater NEC G3; Ampulla of Vater Poorly Differentiated Endocrine Carcinoma; Ampulla of Vater Small and Large Cell Endocrine Carcinoma; Neuroendocrine Carcinoma of the Ampullary Region

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the ampulla of Vater and the periampullary region. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. Patients usually present with advanced stage disease. [from NCI]

Professional guidelines

PubMed

Komiyama S, Okusaka T, Maruki Y, Ohba A, Nagashio Y, Kondo S, Hijioka S, Morizane C, Ueno H, Sukeda A, Mizui T, Takamoto T, Nara S, Ban D, Esaki M, Hiraoka N, Shimada K
Intern Med 2024 Apr 1;63(7):891-901. Epub 2023 Aug 23 doi: 10.2169/internalmedicine.2016-23. PMID: 37612088Free PMC Article
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Mahansaria SS, Agrawal N, Arora A, Bihari C, Appukuttan M, Chattopadhyay TK
Int J Surg Pathol 2017 Oct;25(7):585-591. Epub 2017 May 29 doi: 10.1177/1066896917712454. PMID: 28552015

Recent clinical studies

Etiology

Yamamoto K, Itoi T, Iwasaki E, Tsuchiya T, Ishii K, Tonozuka R, Mukai S, Nagai K, Kitagawa Y
J Hepatobiliary Pancreat Sci 2022 Oct;29(10):1142-1150. Epub 2021 Dec 6 doi: 10.1002/jhbp.1084. PMID: 34826213
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Chapman BC, Gleisner A, Ibrahim-Zada I, Overbey DM, Paniccia A, Meguid C, Brauer B, Gajdos C, McCarter MD, Schulick RD, Edil BH
Surg Endosc 2018 Feb;32(2):915-922. Epub 2017 Aug 4 doi: 10.1007/s00464-017-5766-8. PMID: 28779245
Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB
Cancer Cytopathol 2017 May;125(5):332-340. Epub 2017 Mar 3 doi: 10.1002/cncy.21841. PMID: 28257167Free PMC Article
Adham M, Singhirunnusorn J
Eur J Surg Oncol 2012 Apr;38(4):340-5. Epub 2012 Jan 20 doi: 10.1016/j.ejso.2011.12.015. PMID: 22264964

Diagnosis

Yamamoto K, Itoi T, Iwasaki E, Tsuchiya T, Ishii K, Tonozuka R, Mukai S, Nagai K, Kitagawa Y
J Hepatobiliary Pancreat Sci 2022 Oct;29(10):1142-1150. Epub 2021 Dec 6 doi: 10.1002/jhbp.1084. PMID: 34826213
Trindade AJ, Benias PC, Goldenberg S
Clin Gastroenterol Hepatol 2021 Mar;19(3):e24-e25. Epub 2020 Jan 15 doi: 10.1016/j.cgh.2020.01.010. PMID: 31954200
Attila T, Parlak E, Alper E, Dişibeyaz S, Çiçek B, Ödemiş B
Turk J Gastroenterol 2018 May;29(3):325-334. doi: 10.5152/tjg.2018.17378. PMID: 29755017Free PMC Article
Jang SK, Kim JH, Joo I, Jeon JH, Shin KS, Han JK, Choi BI
Eur Radiol 2015 Oct;25(10):2880-8. Epub 2015 Apr 28 doi: 10.1007/s00330-015-3721-4. PMID: 25916385
Snady H
Hematol Oncol Clin North Am 2002 Feb;16(1):53-79. doi: 10.1016/s0889-8588(01)00010-7. PMID: 12063829

Therapy

Wen LJ, Chen JH, Xu HJ, Yu Q, Deng Y, Liu K
Medicine (Baltimore) 2020 Dec 11;99(50):e23271. doi: 10.1097/MD.0000000000023271. PMID: 33327249Free PMC Article
Odufalu FD, Zubairu J, Silverman W
BMJ Case Rep 2018 Sep 4;2018 doi: 10.1136/bcr-2017-221895. PMID: 30181397Free PMC Article
Taghizadeh-Hesary F, Moradi A, Malekzadeh M
BMJ Case Rep 2018 Jun 27;2018 doi: 10.1136/bcr-2018-224845. PMID: 29950363Free PMC Article
Adham M, Singhirunnusorn J
Eur J Surg Oncol 2012 Apr;38(4):340-5. Epub 2012 Jan 20 doi: 10.1016/j.ejso.2011.12.015. PMID: 22264964
Snady H
Hematol Oncol Clin North Am 2002 Feb;16(1):53-79. doi: 10.1016/s0889-8588(01)00010-7. PMID: 12063829

Prognosis

Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Chapman BC, Gleisner A, Ibrahim-Zada I, Overbey DM, Paniccia A, Meguid C, Brauer B, Gajdos C, McCarter MD, Schulick RD, Edil BH
Surg Endosc 2018 Feb;32(2):915-922. Epub 2017 Aug 4 doi: 10.1007/s00464-017-5766-8. PMID: 28779245
Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB
Cancer Cytopathol 2017 May;125(5):332-340. Epub 2017 Mar 3 doi: 10.1002/cncy.21841. PMID: 28257167Free PMC Article
Beggs RE, Kelly ME, Eltayeb O, Crotty P, McDermott R, Ridgway PF
JOP 2012 Sep 10;13(5):470-5. doi: 10.6092/1590-8577/821. PMID: 22964952
Snady H
Hematol Oncol Clin North Am 2002 Feb;16(1):53-79. doi: 10.1016/s0889-8588(01)00010-7. PMID: 12063829

Clinical prediction guides

Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Yang S, Lu J, Cai Y, Li B, Xiong X
BMC Gastroenterol 2021 Aug 25;21(1):329. doi: 10.1186/s12876-021-01909-z. PMID: 34433421Free PMC Article
Nappo G, Gentile D, Galvanin J, Capretti G, Ridolfi C, Petitti T, Spaggiari P, Carrara S, Gavazzi F, Repici A, Zerbi A
Surg Endosc 2020 Oct;34(10):4358-4368. Epub 2019 Oct 23 doi: 10.1007/s00464-019-07206-x. PMID: 31646438
Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB
Cancer Cytopathol 2017 May;125(5):332-340. Epub 2017 Mar 3 doi: 10.1002/cncy.21841. PMID: 28257167Free PMC Article
Jang SK, Kim JH, Joo I, Jeon JH, Shin KS, Han JK, Choi BI
Eur Radiol 2015 Oct;25(10):2880-8. Epub 2015 Apr 28 doi: 10.1007/s00330-015-3721-4. PMID: 25916385

Recent systematic reviews

Saito K, Matsuo Y, Denda Y, Nonoyama K, Murase H, Kato T, Hayashi Y, Imafuji H, Morimoto M, Ogawa R, Takahashi H, Takiguchi S
J Med Case Rep 2023 Mar 28;17(1):127. doi: 10.1186/s13256-023-03828-x. PMID: 36973791Free PMC Article
Ziogas IA, Rallis KS, Tasoudis PT, Moris D, Schulick RD, Del Chiaro M
Eur J Surg Oncol 2023 Apr;49(4):682-687. Epub 2023 Jan 9 doi: 10.1016/j.ejso.2023.01.005. PMID: 36646615
Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044
Wen LJ, Chen JH, Xu HJ, Yu Q, Deng Y, Liu K
Medicine (Baltimore) 2020 Dec 11;99(50):e23271. doi: 10.1097/MD.0000000000023271. PMID: 33327249Free PMC Article

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