U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Ampulla of Vater Undifferentiated Carcinoma

MedGen UID:
231059
•Concept ID:
C1332254
•
Neoplastic Process

Definition

An aggressive carcinoma arising from the ampulla of Vater. Morphologically, it is characterized by the presence of malignant epithelial cells without evidence of glandular or squamous differentiation. Signs and symptoms include jaundice, abdominal pain, anorexia, nausea, vomiting, and weight loss. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpulla of Vater Undifferentiated Carcinoma

Professional guidelines

PubMed

The Diagnosis and Treatment of Ampullary Carcinoma.
Walter D, Schnitzbauer AA, Schulze F, Trojan J
Dtsch Arztebl Int 2023 Oct 27;120(43):729-735. doi: 10.3238/arztebl.m2023.0195. PMID: 37656482Free PMC Article
Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.
Chiorean EG, Chiaro MD, Tempero MA, Malafa MP, Benson AB, Cardin DB, Christensen JA, Chung V, Czito B, Dillhoff M, Donahue TR, Dotan E, Fountzilas C, Glazer ES, Hardacre J, Hawkins WG, Klute K, Ko AH, Kunstman JW, LoConte N, Lowy AM, Masood A, Moravek C, Nakakura EK, Narang AK, Nardo L, Obando J, Polanco PM, Reddy S, Reyngold M, Scaife C, Shen J, Truty MJ, Vollmer C, Wolff RA, Wolpin BM, Rn BM, Lubin S, Darlow SD
J Natl Compr Canc Netw 2023 Jul;21(7):753-782. doi: 10.6004/jnccn.2023.0034. PMID: 37433437
Controversies in the diagnosis and treatment of periampullary tumours.
Skórzewska M, Kurzawa P, Ciszewski T, Pelc Z, Polkowski WP
Surg Oncol 2022 Sep;44:101853. Epub 2022 Sep 14 doi: 10.1016/j.suronc.2022.101853. PMID: 36152420

Recent clinical studies

Etiology

Impact of histopathological type on the prognosis of ampullary carcinoma: A systematic review and meta-analysis.
Shin DW, Kim S, Jung K, Jung JH, Kim B, Ahn J, Kim J, Hwang JH, Lee JC
Eur J Surg Oncol 2023 Feb;49(2):306-315. Epub 2022 Oct 9 doi: 10.1016/j.ejso.2022.10.001. PMID: 36272870
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
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
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Laparoscopic pancreatoduodenectoy.
Guerrero-Martínez GA, Estrada-Gómez R, Basilio-Roque A, Viveros-Luna R, Lorenzo-Yacamix C, Dávila-Esparza PA
Cir Cir 2020;88(3):263-268. doi: 10.24875/CIRU.19000093. PMID: 32539015
Ampullary cancer: an overview.
Ahn DH, Bekaii-Saab T
Am Soc Clin Oncol Educ Book 2014:112-5. doi: 10.14694/EdBook_AM.2014.34.112. PMID: 24857067Free PMC Article

Diagnosis

Enteroblastic adenocarcinoma of the ampulla of Vater.
Rodríguez-Villena A, Veliz-Domínguez A, González-García I, Ruz-Caracuel I
Rev Esp Patol 2024 Apr-Jun;57(2):151-155. Epub 2024 Mar 7 doi: 10.1016/j.patol.2024.01.004. PMID: 38599738
The Diagnosis and Treatment of Ampullary Carcinoma.
Walter D, Schnitzbauer AA, Schulze F, Trojan J
Dtsch Arztebl Int 2023 Oct 27;120(43):729-735. doi: 10.3238/arztebl.m2023.0195. PMID: 37656482Free PMC Article
Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology.
Chiorean EG, Chiaro MD, Tempero MA, Malafa MP, Benson AB, Cardin DB, Christensen JA, Chung V, Czito B, Dillhoff M, Donahue TR, Dotan E, Fountzilas C, Glazer ES, Hardacre J, Hawkins WG, Klute K, Ko AH, Kunstman JW, LoConte N, Lowy AM, Masood A, Moravek C, Nakakura EK, Narang AK, Nardo L, Obando J, Polanco PM, Reddy S, Reyngold M, Scaife C, Shen J, Truty MJ, Vollmer C, Wolff RA, Wolpin BM, Rn BM, Lubin S, Darlow SD
J Natl Compr Canc Netw 2023 Jul;21(7):753-782. doi: 10.6004/jnccn.2023.0034. PMID: 37433437
Controversies in the diagnosis and treatment of periampullary tumours.
Skórzewska M, Kurzawa P, Ciszewski T, Pelc Z, Polkowski WP
Surg Oncol 2022 Sep;44:101853. Epub 2022 Sep 14 doi: 10.1016/j.suronc.2022.101853. PMID: 36152420
Treatment Approach to Adenocarcinoma of the Ampulla of Vater.
Patel M, Uboha NV
Curr Treat Options Oncol 2021 Sep 29;22(11):103. doi: 10.1007/s11864-021-00894-5. PMID: 34586537

Therapy

Ampullary carcinoma of the duodenum: current clinical issues and genomic overview.
Okano K, Oshima M, Suto H, Ando Y, Asano E, Kamada H, Kobara H, Masaki T, Suzuki Y
Surg Today 2022 Feb;52(2):189-197. Epub 2021 Apr 2 doi: 10.1007/s00595-021-02270-0. PMID: 33797636
Treatment Approach to Adenocarcinoma of the Ampulla of Vater.
Patel M, Uboha NV
Curr Treat Options Oncol 2021 Sep 29;22(11):103. doi: 10.1007/s11864-021-00894-5. PMID: 34586537
Ampullary cancer: an overview.
Ahn DH, Bekaii-Saab T
Am Soc Clin Oncol Educ Book 2014:112-5. doi: 10.14694/EdBook_AM.2014.34.112. PMID: 24857067Free PMC Article
Effect of adjuvant chemotherapy with fluorouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma: the ESPAC-3 periampullary cancer randomized trial.
Neoptolemos JP, Moore MJ, Cox TF, Valle JW, Palmer DH, McDonald AC, Carter R, Tebbutt NC, Dervenis C, Smith D, Glimelius B, Charnley RM, Lacaine F, Scarfe AG, Middleton MR, Anthoney A, Ghaneh P, Halloran CM, Lerch MM, Oláh A, Rawcliffe CL, Verbeke CS, Campbell F, Büchler MW; European Study Group for Pancreatic Cancer
JAMA 2012 Jul 11;308(2):147-56. doi: 10.1001/jama.2012.7352. PMID: 22782416
Bile duct malignancies.
Tucek S, Tomasek J, Halámkova J, Kiss I, Andrasina T, Hemmelová B, Adámková-Krákorová D, Vyzula R
Klin Onkol 2010;23(4):231-41. PMID: 20806821

Prognosis

The Diagnosis and Treatment of Ampullary Carcinoma.
Walter D, Schnitzbauer AA, Schulze F, Trojan J
Dtsch Arztebl Int 2023 Oct 27;120(43):729-735. doi: 10.3238/arztebl.m2023.0195. PMID: 37656482Free PMC Article
Ampullary carcinoma of the duodenum: current clinical issues and genomic overview.
Okano K, Oshima M, Suto H, Ando Y, Asano E, Kamada H, Kobara H, Masaki T, Suzuki Y
Surg Today 2022 Feb;52(2):189-197. Epub 2021 Apr 2 doi: 10.1007/s00595-021-02270-0. PMID: 33797636
Treatment Approach to Adenocarcinoma of the Ampulla of Vater.
Patel M, Uboha NV
Curr Treat Options Oncol 2021 Sep 29;22(11):103. doi: 10.1007/s11864-021-00894-5. PMID: 34586537
Ampullary cancer: an overview.
Ahn DH, Bekaii-Saab T
Am Soc Clin Oncol Educ Book 2014:112-5. doi: 10.14694/EdBook_AM.2014.34.112. PMID: 24857067Free PMC Article
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

Ampullary Cancer: Histological Subtypes, Markers, and Clinical Behaviour-State of the Art and Perspectives.
Nappo G, Funel N, Laurenti V, Stenner E, Carrara S, Bozzarelli S, Spaggiari P, Zerbi A
Curr Oncol 2023 Jul 22;30(7):6996-7006. doi: 10.3390/curroncol30070507. PMID: 37504367Free PMC Article
Ampullary carcinoma of the duodenum: current clinical issues and genomic overview.
Okano K, Oshima M, Suto H, Ando Y, Asano E, Kamada H, Kobara H, Masaki T, Suzuki Y
Surg Today 2022 Feb;52(2):189-197. Epub 2021 Apr 2 doi: 10.1007/s00595-021-02270-0. PMID: 33797636
Approaches to Biopsy and Resection Specimens from the Ampulla.
Xue Y, Reid MD
Surg Pathol Clin 2020 Sep;13(3):453-467. Epub 2020 Jul 15 doi: 10.1016/j.path.2020.05.005. PMID: 32773194
Effect of adjuvant chemotherapy with fluorouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma: the ESPAC-3 periampullary cancer randomized trial.
Neoptolemos JP, Moore MJ, Cox TF, Valle JW, Palmer DH, McDonald AC, Carter R, Tebbutt NC, Dervenis C, Smith D, Glimelius B, Charnley RM, Lacaine F, Scarfe AG, Middleton MR, Anthoney A, Ghaneh P, Halloran CM, Lerch MM, Oláh A, Rawcliffe CL, Verbeke CS, Campbell F, Büchler MW; European Study Group for Pancreatic Cancer
JAMA 2012 Jul 11;308(2):147-56. doi: 10.1001/jama.2012.7352. PMID: 22782416
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

The clinical implication of minimally invasive versus open pancreatoduodenectomy for non-pancreatic periampullary cancer: a systematic review and individual patient data meta-analysis.
Uijterwijk BA, Kasai M, Lemmers DHL, Chinnusamy P, van Hilst J, Ielpo B, Wei K, Song KB, Kim SC, Klompmaker S, Jang JY, Herremans KM, Bencini L, Coratti A, Mazzola M, Menon KV, Goh BKP, Qin R, Besselink MG, Abu Hilal M; International Study Group on non-pancreatic periAmpullary CAncer (ISGACA)
Langenbecks Arch Surg 2023 Aug 15;408(1):311. doi: 10.1007/s00423-023-03047-4. PMID: 37581763Free PMC Article
Management and outcomes of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A systematic review and pooled analysis of 56 patients.
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
Impact of histopathological type on the prognosis of ampullary carcinoma: A systematic review and meta-analysis.
Shin DW, Kim S, Jung K, Jung JH, Kim B, Ahn J, Kim J, Hwang JH, Lee JC
Eur J Surg Oncol 2023 Feb;49(2):306-315. Epub 2022 Oct 9 doi: 10.1016/j.ejso.2022.10.001. PMID: 36272870
Somatostatinoma of the Ampulla of Vater: A Systematic Review.
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
Review of the investigation and surgical management of resectable ampullary adenocarcinoma.
Askew J, Connor S
HPB (Oxford) 2013 Nov;15(11):829-38. Epub 2013 Jan 10 doi: 10.1111/hpb.12038. PMID: 23458317Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...