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Ampulla of vater small cell neuroendocrine carcinoma

MedGen UID:
233974
•Concept ID:
C1332250
•
Neoplastic Process
Synonyms: Ampulla of Vater Small Cell Carcinoma; ampulla of Vater small cell carcinoma; Ampulla of Vater Small Cell NEC; ampulla of Vater small cell NEC; Ampulla of Vater Small Cell Neuroendocrine Carcinoma; ampulla of vater small cell neuroendocrine carcinoma; ampulla of Vater small cell neuroendocrine carcinoma; Ampullary Small Cell Carcinoma; ampullary small cell carcinoma; hepatopancreatic ampulla small cell carcinoma; Small Cell Neuroendocrine Carcinoma of the Ampullary Region; small cell neuroendocrine carcinoma of the ampullary region
 
Monarch Initiative: MONDO:0004117

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the ampulla of Vater and the periampullary region. It is characterized by the presence of malignant small cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpulla of vater small cell neuroendocrine carcinoma

Professional guidelines

PubMed

Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
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

Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
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
HepPar-1 and Arginase-1 Immunohistochemistry in Adenocarcinoma of the Small Intestine and Ampullary Region.
Lagana S, Hsiao S, Bao F, Sepulveda A, Moreira R, Lefkowitch J, Remotti H
Arch Pathol Lab Med 2015 Jun;139(6):791-5. doi: 10.5858/arpa.2013-0249-OA. PMID: 26030248
Laparoscopic pancreaticoduodenectomy to treat cancer of the ampulla of Vater.
Lu B, Cai X, Lu W, Huang Y, Jin X
JSLS 2006 Jan-Mar;10(1):97-100. PMID: 16709370Free PMC Article
Signet-ring cell carcinoma of the ampulla of Vater.
Li L, Chen QH, Sullivan JD, Breuer FU
Ann Clin Lab Sci 2004 Autumn;34(4):471-5. PMID: 15648791
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

Diagnosis

Neuroendocrine carcinoma of the minor papilla with pancreas divisum: a case report and review of the literature.
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
Neuroendocrine tumor of the ampulla of Vater showing chronological endoscopic changes.
Fujii M, Okamoto Y, Fujioka S, Yoshioka M, Shiode J
Clin J Gastroenterol 2022 Feb;15(1):205-209. Epub 2022 Feb 3 doi: 10.1007/s12328-021-01553-w. PMID: 35113343
Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
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
HepPar-1 and Arginase-1 Immunohistochemistry in Adenocarcinoma of the Small Intestine and Ampullary Region.
Lagana S, Hsiao S, Bao F, Sepulveda A, Moreira R, Lefkowitch J, Remotti H
Arch Pathol Lab Med 2015 Jun;139(6):791-5. doi: 10.5858/arpa.2013-0249-OA. PMID: 26030248
Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater.
Sun JH, Chao M, Zhang SZ, Zhang GQ, Li B, Wu JJ
World J Gastroenterol 2008 Aug 7;14(29):4709-12. doi: 10.3748/wjg.14.4709. PMID: 18698690Free PMC Article

Therapy

Molecular Characterization of Biliary Tract Cancer Predicts Chemotherapy and Programmed Death 1/Programmed Death-Ligand 1 Blockade Responses.
Yoon JG, Kim MH, Jang M, Kim H, Hwang HK, Kang CM, Lee WJ, Kang B, Lee CK, Lee MG, Chung HC, Choi HJ, Park YN
Hepatology 2021 Oct;74(4):1914-1931. Epub 2021 Jul 29 doi: 10.1002/hep.31862. PMID: 33884649
Two-stage operation for synchronous triple primary cancer of the esophagus, stomach, and ampulla of Vater: report of a case.
Fukaya M, Abe T, Yokoyama Y, Itatsu K, Nagino M
Surg Today 2014 May;44(5):967-71. Epub 2013 Mar 17 doi: 10.1007/s00595-013-0549-x. PMID: 23504004
Cisplatin and etoposide as first-line chemotherapy for poorly differentiated neuroendocrine carcinoma of the hepatobiliary tract and pancreas.
Iwasa S, Morizane C, Okusaka T, Ueno H, Ikeda M, Kondo S, Tanaka T, Nakachi K, Mitsunaga S, Kojima Y, Hagihara A, Hiraoka N
Jpn J Clin Oncol 2010 Apr;40(4):313-8. Epub 2010 Jan 4 doi: 10.1093/jjco/hyp173. PMID: 20047862
Primary small cell carcinoma of the common bile duct, in which surgical treatment was performed after neoadjuvant chemotherapy: report of a case.
Hazama K, Suzuki Y, Takahashi M, Takahashi Y, Yoshioka T, Takano S, Kondoh S, Katoh H
Surg Today 2003;33(11):870-2. doi: 10.1007/s00595-003-2594-3. PMID: 14605962
An immunohistochemical and ultrastructural study of case of small-cell neuroendocrine carcinoma in the ampullary region of the duodenum.
Sarker AB, Hoshida Y, Akagi S, Hayashi K, Murakami I, Jeon HJ, Takahashi K, Akagi T
Acta Pathol Jpn 1992 Jul;42(7):529-35. doi: 10.1111/j.1440-1827.1992.tb03101.x. PMID: 1384272

Prognosis

Neuroendocrine carcinoma of the minor papilla with pancreas divisum: a case report and review of the literature.
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
Molecular Characterization of Biliary Tract Cancer Predicts Chemotherapy and Programmed Death 1/Programmed Death-Ligand 1 Blockade Responses.
Yoon JG, Kim MH, Jang M, Kim H, Hwang HK, Kang CM, Lee WJ, Kang B, Lee CK, Lee MG, Chung HC, Choi HJ, Park YN
Hepatology 2021 Oct;74(4):1914-1931. Epub 2021 Jul 29 doi: 10.1002/hep.31862. PMID: 33884649
Establishment of Patient-Derived Organoids and Drug Screening for Biliary Tract Carcinoma.
Saito Y, Muramatsu T, Kanai Y, Ojima H, Sukeda A, Hiraoka N, Arai E, Sugiyama Y, Matsuzaki J, Uchida R, Yoshikawa N, Furukawa R, Saito H
Cell Rep 2019 Apr 23;27(4):1265-1276.e4. doi: 10.1016/j.celrep.2019.03.088. PMID: 31018139
Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
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
Periampullary carcinomas.
Filiz G, Yerci O, Adim SB, Gurel S, Dolar E, Memik F
Hepatogastroenterology 2007 Jun;54(76):1247-9. PMID: 17629080

Clinical prediction guides

Pattern of placental alkaline phosphatase (PLAP) expression in human tumors: a tissue microarray study on 12,381 tumors.
Reiswich V, Gorbokon N, Luebke AM, Burandt E, Menz A, Kluth M, Hube-Magg C, Wittmer C, Weidemann S, Fraune C, Möller K, Lebok P, Sauter G, Simon R, Uhlig R, Wilczak W, Jacobsen F, Minner S, Krech R, Bernreuther C, Marx A, Steurer S, Clauditz T, Krech T
J Pathol Clin Res 2021 Nov;7(6):577-589. Epub 2021 Aug 7 doi: 10.1002/cjp2.237. PMID: 34363325Free PMC Article
Molecular Characterization of Biliary Tract Cancer Predicts Chemotherapy and Programmed Death 1/Programmed Death-Ligand 1 Blockade Responses.
Yoon JG, Kim MH, Jang M, Kim H, Hwang HK, Kang CM, Lee WJ, Kang B, Lee CK, Lee MG, Chung HC, Choi HJ, Park YN
Hepatology 2021 Oct;74(4):1914-1931. Epub 2021 Jul 29 doi: 10.1002/hep.31862. PMID: 33884649
MMR Deficiency is Homogeneous in Pancreatic Carcinoma and Associated with High Density of Cd8-Positive Lymphocytes.
Fraune C, Burandt E, Simon R, Hube-Magg C, Makrypidi-Fraune G, Kluth M, Büscheck F, Höflmayer D, Blessin NC, Mandelkow T, Li W, Perez D, Izbicki JR, Wilczak W, Sauter G, Schrader J, Neipp M, Mofid H, Daniels T, Isbert C, Clauditz TS, Steurer S
Ann Surg Oncol 2020 Oct;27(10):3997-4006. Epub 2020 Feb 27 doi: 10.1245/s10434-020-08209-y. PMID: 32108923Free PMC Article
Inhibin-expressing clear cell neuroendocrine tumor of the ampulla: an unusual presentation of von Hippel-Lindau disease.
Gucer H, Szentgyorgyi E, Ezzat S, Asa SL, Mete O
Virchows Arch 2013 Oct;463(4):593-7. Epub 2013 Aug 4 doi: 10.1007/s00428-013-1465-6. PMID: 23913169
Periampullary carcinomas.
Filiz G, Yerci O, Adim SB, Gurel S, Dolar E, Memik F
Hepatogastroenterology 2007 Jun;54(76):1247-9. PMID: 17629080

Recent systematic reviews

Neuroendocrine carcinoma of the minor papilla with pancreas divisum: a case report and review of the literature.
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

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