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Small Intestinal Signet Ring Cell Carcinoma

MedGen UID:
313710
•Concept ID:
C1710110
•
Neoplastic Process
Synonym: Small Intestinal Signet-Ring Cell Carcinoma

Definition

An invasive adenocarcinoma that arises from the small intestine. It is characterized by the presence of malignant glandular cells in which the nucleus is pressed to one side by the presence of intracytoplasmic mucus. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall Intestinal Signet Ring Cell Carcinoma

Recent clinical studies

Etiology

Signet-ring cell carcinoma of the duodenal bulb presenting with gastrointestinal hemorrhage: a case report and literature review.
Ye N, Bao X, Zhao X, Wang B
BMC Gastroenterol 2022 May 9;22(1):226. doi: 10.1186/s12876-022-02267-0. PMID: 35534806Free PMC Article
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
Survival in signet ring cell carcinoma varies based on primary tumor location: a Surveillance, Epidemiology, and End Results database analysis.
Wu SG, Chen XT, Zhang WW, Sun JY, Li FY, He ZY, Pei XQ, Lin Q
Expert Rev Gastroenterol Hepatol 2018 Feb;12(2):209-214. Epub 2017 Dec 17 doi: 10.1080/17474124.2018.1416291. PMID: 29227748
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
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

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: 37682279Free PMC Article
Superficial nonampullary duodenal epithelial tumor: a rare case with signet-ring cell carcinoma component diagnosed by magnifying endoscopy.
Muramoto T, Ohata K, Takayanagi S, Kimoto Y, Ishii R, Negishi R, Minato Y
Endoscopy 2023 Dec;55(S 01):E90-E91. Epub 2022 Oct 10 doi: 10.1055/a-1931-4105. PMID: 36216257Free PMC Article
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
Krukenberg tumor: metastasis of Meckel's diverticular adenocarcinoma to ovaries.
Sakpal SV, Babel N, Pulinthanathu R, Denehy TR, Chamberlain RS
J Nippon Med Sch 2009 Apr;76(2):96-102. doi: 10.1272/jnms.76.96. PMID: 19443995

Therapy

A case of small bowel adenocarcinoma wherein nivolumab conferred temporary benefit in disease control.
Iijima K, Oozeki M, Ikeda K, Honda H, Ishibashi H, Yamaoka M, Fujieda S, Saitoh H, Goto M, Araki M, Amagai K
Clin J Gastroenterol 2020 Jun;13(3):372-376. Epub 2019 Nov 7 doi: 10.1007/s12328-019-01064-9. PMID: 31701367
Occurrence of signet-ring cell carcinoma with cholangiocarcinoma 25 years after choledochal cyst excision: A case report.
Zhang C, Zhou J, Kou K, Liu S, We F, Wang G
Medicine (Baltimore) 2018 Feb;97(8):e9956. doi: 10.1097/MD.0000000000009956. PMID: 29465585Free PMC Article
Outcomes of screening gastroscopy in first-degree relatives of patients fulfilling hereditary diffuse gastric cancer criteria.
van der Post RS, van Dieren J, Grelack A, Hoogerbrugge N, van der Kolk LE, Snaebjornsson P, Lansdorp-Vogelaar I, van Krieken JH, Bisseling TM, Cats A
Gastrointest Endosc 2018 Feb;87(2):397-404.e2. Epub 2017 Apr 25 doi: 10.1016/j.gie.2017.04.016. PMID: 28455161
Complications after proximal gastrectomy with jejunal pouch interposition: report of a case.
Katsube T, Konno S, Hamaguchi K, Shimakawa T, Naritaka Y, Ogawa K
Eur J Surg Oncol 2005 Nov;31(9):1036-8. Epub 2005 Sep 9 doi: 10.1016/j.ejso.2005.07.009. PMID: 16154312
Signet ring cell carcinoma in adenomatous polyp at site of ureterosigmoidostomy 16 years after conversion to ileal conduit.
Sohn M, Füzesi L, Deutz F, Lagrange W, Kirkpatrick JC, Braun JC
J Urol 1990 Apr;143(4):805-7. doi: 10.1016/s0022-5347(17)40102-9. PMID: 2156089

Prognosis

Metastatic Signet Ring Cell Carcinoma of the Ampulla of Vater with an Unusually Prolonged Survival.
Javvaji C, Bledsoe JR, Hutchinson L, Cosar EF, McIntosh LJ, Bathini VG
J Gastrointest Cancer 2020 Mar;51(1):354-358. doi: 10.1007/s12029-019-00297-8. PMID: 31456115
Metastatic Ovarian Tumors Originating From a Small Bowel Adenocarcinoma - A Case Report and Brief Literature Review.
Mitsushita J, Netsu S, Suzuki K, Nokubi M, Tanaka A
Int J Gynecol Pathol 2017 May;36(3):253-260. doi: 10.1097/PGP.0000000000000314. PMID: 27513076
Signet Ring Cell Carcinoma of the Ampulla of Vater with Early Development of Bone Metastasis: Case Report and Review of the Rare Malignancy.
Damania R, Weaver J, Cocieru A
J Gastrointest Cancer 2016 Mar;47(1):89-92. doi: 10.1007/s12029-015-9718-z. PMID: 25911029
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
Signet ring cell carcinoma of the ampulla of Vater with leptomeningeal metastases: a case report.
Paplomata E, Wilfong L
J Clin Oncol 2011 Jul 20;29(21):e627-9. Epub 2011 May 23 doi: 10.1200/JCO.2011.35.2385. PMID: 21606434

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
Analysis of clinical and histopathological findings in Russell body gastritis and duodenitis.
Altindag SD, Cakir E, Ekinci N, Avci A, Dilek FH
Ann Diagn Pathol 2019 Jun;40:66-71. Epub 2019 Apr 3 doi: 10.1016/j.anndiagpath.2019.04.003. PMID: 31031217
Intramucosal colorectal carcinoma with invasion of the lamina propria: a study by the Japanese Society for Cancer of the Colon and Rectum.
Kojima M, Shimazaki H, Iwaya K, Nakamura T, Kawachi H, Ichikawa K, Sekine S, Ishiguro S, Shimoda T, Kushima R, Yao T, Fujimori T, Hase K, Watanabe T, Sugihara K, Lauwers GY, Ochiai A
Hum Pathol 2017 Aug;66:230-237. Epub 2017 Jul 13 doi: 10.1016/j.humpath.2017.04.031. PMID: 28711649
Metastatic Ovarian Tumors Originating From a Small Bowel Adenocarcinoma - A Case Report and Brief Literature Review.
Mitsushita J, Netsu S, Suzuki K, Nokubi M, Tanaka A
Int J Gynecol Pathol 2017 May;36(3):253-260. doi: 10.1097/PGP.0000000000000314. PMID: 27513076
Loss of ARID1A expression is associated with poor prognosis in small intestinal carcinoma.
Kim MJ, Gu MJ, Chang HK, Yu E
Histopathology 2015 Mar;66(4):508-16. Epub 2015 Jan 12 doi: 10.1111/his.12566. PMID: 25400081

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