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Ampullary signet ring cell adenocarcinoma

MedGen UID:
231057
•Concept ID:
C1332249
•
Neoplastic Process
Synonyms: Ampulla of Vater Signet Ring Cell Carcinoma; ampulla of Vater signet Ring cell carcinoma; Ampulla of Vater Signet-Ring Cell Carcinoma; ampullary signet ring cell adenocarcinoma; Ampullary Signet Ring Cell Carcinoma; ampullary signet Ring cell carcinoma; ampullary signet ring cell carcinoma
 
Monarch Initiative: MONDO:0002669

Definition

An adenocarcinoma arising from the ampulla of Vater. Morphologically, it is characterized by the presence of mucin-containing signet-ring cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAmpullary signet ring cell adenocarcinoma

Recent clinical studies

Etiology

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
Synchronous signet-ring cell carcinoma of the duodenum and ampullary intestinal-type carcinoma.
Aurello P, Petrucciani N, D'Angelo F, Nigri G, Picchetto A, Cicchini C, Maglio R, Ramacciato G
Am Surg 2011 Dec;77(12):E262-4. PMID: 22273189
Gastric and pancreatoduodenal resection for malignant lesions after previous gastric bypass--diagnosis and methods of reconstruction.
Swain JM, Adams RB, Farnell MB, Que FG, Sarr MG
Surg Obes Relat Dis 2010 Nov-Dec;6(6):670-5. Epub 2010 Apr 1 doi: 10.1016/j.soard.2010.03.287. PMID: 20627707
Signet-ring cell carcinoma of the ampulla of Vater: report of a case.
Akatsu T, Aiura K, Takahashi S, Kameyama K, Kitajima M, Kitagawa Y
Surg Today 2007;37(12):1110-4. Epub 2007 Nov 26 doi: 10.1007/s00595-007-3534-4. PMID: 18030577
Carcinoma of the ampulla of Vater: comparative histologic/immunohistochemical classification and follow-up.
Zhou H, Schaefer N, Wolff M, Fischer HP
Am J Surg Pathol 2004 Jul;28(7):875-82. doi: 10.1097/00000478-200407000-00005. PMID: 15223956

Diagnosis

Pseudoachalasia as the first manifestation of signet-ring cell duodenal carcinoma.
Trad N, Bel Hadj Mabrouk E, Mensi A, Zaimi Y, Ksantini M, Said Y
Tunis Med 2023 Feb 12;101(2):317-320. PMID: 37682279
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
Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States.
Kinslow CJ, May MS, Kozak M, Pollom EL, Chang DT
HPB (Oxford) 2020 Dec;22(12):1759-1765. Epub 2020 Apr 18 doi: 10.1016/j.hpb.2020.03.024. PMID: 32317226
Signet-ring cell carcinoma of the ampulla of Vater: a case diagnosed via repeated biopsies.
Ikeda C, Makino N, Matsuda A, Kakizaki Y, Ishizawa T, Kobayashi T, Sugahara S, Nishiduka M, Tsunoda M, Haga J, Tsunoda R, Ueno Y
Clin J Gastroenterol 2020 Aug;13(4):607-614. Epub 2020 Jan 24 doi: 10.1007/s12328-020-01097-5. PMID: 31981088Free PMC Article
A rare case of signet-ring cell carcinoma associated with poorly differentiated adenocarcinoma of the non-ampullary duodenum.
Caraşca C, Simion G, Nechifor-Boilă AC, Boeriu AM, Dobru ED
Rom J Morphol Embryol 2018;59(1):311-315. PMID: 29940643

Therapy

Feasibility study of postoperative adjuvant chemotherapy with S-1 in patients with biliary tract cancer.
Nakachi K, Konishi M, Ikeda M, Shimada K, Okusaka T, Saiura A, Ishii H, Sugiyama M, Furuse J, Sakamoto H, Shimamura T, Ohta T
Int J Clin Oncol 2018 Oct;23(5):894-899. Epub 2018 Apr 28 doi: 10.1007/s10147-018-1283-6. PMID: 29705976
Poorly differentiated adenocarcinoma with signet-ring cells of the Vater's ampulla, without jaundice but with disseminated carcinomatosis.
Nabeshima S, Kishihara Y, Nabeshima A, Yamaga S, Kinjo M, Kashiwagi S, Hayashi J
Fukuoka Igaku Zasshi 2003 Jul;94(7):235-40. PMID: 14509231

Prognosis

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
Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States.
Kinslow CJ, May MS, Kozak M, Pollom EL, Chang DT
HPB (Oxford) 2020 Dec;22(12):1759-1765. Epub 2020 Apr 18 doi: 10.1016/j.hpb.2020.03.024. PMID: 32317226
Poorly Cohesive (Signet Ring Cell) Carcinoma of the Ampulla of Vater.
Tuncel D, Basturk O, Bradley KT, Kim GE, Xue Y, Reid MD, Balci S, Erbarut I, Adsay V
Int J Surg Pathol 2020 May;28(3):236-244. Epub 2019 Oct 15 doi: 10.1177/1066896919880968. PMID: 31612756
Signet Ring Cell Carcinoma of the Ampulla of Vater With Focal Neuroendocrine Differentiation of the Amphicrine Type: Report of a Case With Long-Term Survival.
Fornelli A, Zanini N, De Biase D, Lega S, Lombardi R, Masetti M, Jovine E, Fabbri C, Larghi A
Int J Surg Pathol 2019 Feb;27(1):89-93. Epub 2018 Jul 11 doi: 10.1177/1066896918784666. PMID: 29996669
Poorly differentiated adenocarcinoma with signet-ring cells of the Vater's ampulla, without jaundice but with disseminated carcinomatosis.
Nabeshima S, Kishihara Y, Nabeshima A, Yamaga S, Kinjo M, Kashiwagi S, Hayashi J
Fukuoka Igaku Zasshi 2003 Jul;94(7):235-40. PMID: 14509231

Clinical prediction guides

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
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
DNA mismatch repair deficiency in ampullary carcinoma: a morphologic and immunohistochemical study of 54 cases.
Agaram NP, Shia J, Tang LH, Klimstra DS
Am J Clin Pathol 2010 May;133(5):772-80. doi: 10.1309/AJCPGDDE8PLLDRCC. PMID: 20395525
Signet-ring cell carcinoma of the ampulla of Vater: report of a case.
Akatsu T, Aiura K, Takahashi S, Kameyama K, Kitajima M, Kitagawa Y
Surg Today 2007;37(12):1110-4. Epub 2007 Nov 26 doi: 10.1007/s00595-007-3534-4. PMID: 18030577
Immunohistochemical staining in the diagnosis of pancreatobiliary and ampulla of Vater adenocarcinoma: application of CDX2, CK17, MUC1, and MUC2.
Chu PG, Schwarz RE, Lau SK, Yen Y, Weiss LM
Am J Surg Pathol 2005 Mar;29(3):359-67. doi: 10.1097/01.pas.0000149708.12335.6a. PMID: 15725805

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