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Signet ring cell carcinoma

MedGen UID:
61663
•Concept ID:
C0206696
•
Neoplastic Process
Synonyms: Diffuse gastric cancer; Signet cell adenocarcinoma; Signet ring gastric carcinoma
SNOMED CT: Signet ring carcinoma (87737001); Signet ring cell carcinoma (87737001); Signet ring cell adenocarcinoma (87737001)
 
Monarch Initiative: MONDO:0005092

Definition

A usually aggressive, poorly differentiated invasive adenocarcinoma characterized by the presence of malignant glandular cells in which the nucleus is pressed to one side by the presence of intracytoplasmic mucus. It may arise from the stomach, small and large intestine, ampulla of Vater, appendix, gallbladder, pancreas, lung, bladder, breast, and prostate gland. [from NCI]

Professional guidelines

PubMed

Trends, clinicopathological features, surgical treatment patterns and prognoses of early-onset versus late-onset colorectal cancer: A retrospective cohort study on 34067 patients managed from 2000 to 2021 in a Chinese tertiary center.
Gao XH, Li J, Liu LJ, Zheng NX, Zheng K, Mei Z, Bai CG, Zhang W
Int J Surg 2022 Aug;104:106780. Epub 2022 Jul 16 doi: 10.1016/j.ijsu.2022.106780. PMID: 35850466
Hereditary Diffuse Gastric Cancer: Approaches to Screening, Surveillance, and Treatment.
Pilonis ND, Tischkowitz M, Fitzgerald RC, di Pietro M
Annu Rev Med 2021 Jan 27;72:263-280. Epub 2020 Nov 20 doi: 10.1146/annurev-med-051019-103216. PMID: 33217247
Hereditary diffuse gastric cancer: updated clinical guidelines with an emphasis on germline CDH1 mutation carriers.
van der Post RS, Vogelaar IP, Carneiro F, Guilford P, Huntsman D, Hoogerbrugge N, Caldas C, Schreiber KE, Hardwick RH, Ausems MG, Bardram L, Benusiglio PR, Bisseling TM, Blair V, Bleiker E, Boussioutas A, Cats A, Coit D, DeGregorio L, Figueiredo J, Ford JM, Heijkoop E, Hermens R, Humar B, Kaurah P, Keller G, Lai J, Ligtenberg MJ, O'Donovan M, Oliveira C, Pinheiro H, Ragunath K, Rasenberg E, Richardson S, Roviello F, Schackert H, Seruca R, Taylor A, Ter Huurne A, Tischkowitz M, Joe ST, van Dijck B, van Grieken NC, van Hillegersberg R, van Sandick JW, Vehof R, van Krieken JH, Fitzgerald RC
J Med Genet 2015 Jun;52(6):361-74. Epub 2015 May 15 doi: 10.1136/jmedgenet-2015-103094. PMID: 25979631Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Gastric Cancer, 2023

Recent clinical studies

Etiology

Comparison of [(68)Ga]Ga-FAPI and [(18)F]FDG uptake in patients with gastric signet-ring-cell carcinoma: a multicenter retrospective study.
Chen H, Pang Y, Li J, Kang F, Xu W, Meng T, Shang Q, Zhao J, Guan Y, Wu H, Xie F, Wang J, Sun L
Eur Radiol 2023 Feb;33(2):1329-1341. Epub 2022 Aug 17 doi: 10.1007/s00330-022-09084-9. PMID: 35976396
Clinicopathological Features and Survival of Signet-Ring Cell Carcinoma and Mucinous Adenocarcinoma of Right Colon, Left Colon, and Rectum.
Zhu L, Ling C, Xu T, Zhang J, Zhang Y, Liu Y, Fang C, Yang L, Zhuang W, Wang R, Ping J, Wang M
Pathol Oncol Res 2021;27:1609800. Epub 2021 Jul 2 doi: 10.3389/pore.2021.1609800. PMID: 34276258Free PMC Article
Histological and mutational profile of diffuse gastric cancer: current knowledge and future challenges.
Garcia-Pelaez J, Barbosa-Matos R, Gullo I, Carneiro F, Oliveira C
Mol Oncol 2021 Nov;15(11):2841-2867. Epub 2021 May 2 doi: 10.1002/1878-0261.12948. PMID: 33724653Free PMC Article
Hereditary Diffuse Gastric Cancer: Approaches to Screening, Surveillance, and Treatment.
Pilonis ND, Tischkowitz M, Fitzgerald RC, di Pietro M
Annu Rev Med 2021 Jan 27;72:263-280. Epub 2020 Nov 20 doi: 10.1146/annurev-med-051019-103216. PMID: 33217247
Prognostic significance of frequent CLDN18-ARHGAP26/6 fusion in gastric signet-ring cell cancer.
Shu Y, Zhang W, Hou Q, Zhao L, Zhang S, Zhou J, Song X, Zhang Y, Jiang D, Chen X, Wang P, Xia X, Liao F, Yin D, Chen X, Zhou X, Zhang D, Yin S, Yang K, Liu J, Fu L, Zhang L, Wang Y, Zhang J, An Y, Cheng H, Zheng B, Sun H, Zhao Y, Wang Y, Xie D, Ouyang L, Wang P, Zhang W, Qiu M, Fu X, Dai L, He G, Yang H, Cheng W, Yang L, Liu B, Li W, Dong B, Zhou Z, Wei Y, Peng Y, Xu H, Hu J
Nat Commun 2018 Jun 30;9(1):2447. doi: 10.1038/s41467-018-04907-0. PMID: 29961079Free PMC Article

Diagnosis

Goblet cell adenocarcinoma of the appendix: an update and practical approach to diagnosis and grading.
Bell PD, Pai RK
Hum Pathol 2023 Feb;132:183-196. Epub 2022 Jun 9 doi: 10.1016/j.humpath.2022.06.002. PMID: 35691370
The updated WHO classification of digestive system tumours-gastric adenocarcinoma and dysplasia.
Kushima R
Pathologe 2022 Feb;43(1):8-15. Epub 2021 Nov 22 doi: 10.1007/s00292-021-01023-7. PMID: 34807275
Gastric adenocarcinoma of fundic gland type with signet-ring cell carcinoma component: A case report and review of the literature.
Kai K, Satake M, Tokunaga O
World J Gastroenterol 2018 Jul 14;24(26):2915-2920. doi: 10.3748/wjg.v24.i26.2915. PMID: 30018486Free PMC Article
The relevance and implications of signet-ring cell adenocarcinoma of the oesophagus.
Bleaney CW, Barrow M, Hayes S, Ang Y
J Clin Pathol 2018 Mar;71(3):201-206. Epub 2017 Dec 6 doi: 10.1136/jclinpath-2017-204863. PMID: 29212656
Signet-ring cell carcinoma of the stomach: Impact on prognosis and specific therapeutic challenge.
Pernot S, Voron T, Perkins G, Lagorce-Pages C, Berger A, Taieb J
World J Gastroenterol 2015 Oct 28;21(40):11428-38. doi: 10.3748/wjg.v21.i40.11428. PMID: 26523107Free PMC Article

Therapy

Endoscopic surveillance with systematic random biopsy for the early diagnosis of hereditary diffuse gastric cancer: a prospective 16-year longitudinal cohort study.
Lee CYC, Olivier A, Honing J, Lydon AM, Richardson S, O'Donovan M, Tischkowitz M, Fitzgerald RC, di Pietro M
Lancet Oncol 2023 Jan;24(1):107-116. Epub 2022 Dec 9 doi: 10.1016/S1470-2045(22)00700-8. PMID: 36509094
The Molecular Associations of Signet-Ring Cell Carcinoma in Colorectum: Meta-Analysis and System Review.
Liu X, Huang L, Liu M, Wang Z
Medicina (Kaunas) 2022 Jun 21;58(7) doi: 10.3390/medicina58070836. PMID: 35888555Free PMC Article
Intracellular MUC20 variant 2 maintains mitochondrial calcium homeostasis and enhances drug resistance in gastric cancer.
Fu L, Yonemura A, Yasuda-Yoshihara N, Umemoto T, Zhang J, Yasuda T, Uchihara T, Akiyama T, Kitamura F, Yamashita K, Okamoto Y, Bu L, Wei F, Hu X, Liu Y, Ajani JA, Tan P, Baba H, Ishimoto T
Gastric Cancer 2022 May;25(3):542-557. Epub 2022 Feb 15 doi: 10.1007/s10120-022-01283-z. PMID: 35166958
Prognostic significance of frequent CLDN18-ARHGAP26/6 fusion in gastric signet-ring cell cancer.
Shu Y, Zhang W, Hou Q, Zhao L, Zhang S, Zhou J, Song X, Zhang Y, Jiang D, Chen X, Wang P, Xia X, Liao F, Yin D, Chen X, Zhou X, Zhang D, Yin S, Yang K, Liu J, Fu L, Zhang L, Wang Y, Zhang J, An Y, Cheng H, Zheng B, Sun H, Zhao Y, Wang Y, Xie D, Ouyang L, Wang P, Zhang W, Qiu M, Fu X, Dai L, He G, Yang H, Cheng W, Yang L, Liu B, Li W, Dong B, Zhou Z, Wei Y, Peng Y, Xu H, Hu J
Nat Commun 2018 Jun 30;9(1):2447. doi: 10.1038/s41467-018-04907-0. PMID: 29961079Free PMC Article
Signet-ring cell carcinoma of the stomach: Impact on prognosis and specific therapeutic challenge.
Pernot S, Voron T, Perkins G, Lagorce-Pages C, Berger A, Taieb J
World J Gastroenterol 2015 Oct 28;21(40):11428-38. doi: 10.3748/wjg.v21.i40.11428. PMID: 26523107Free PMC Article

Prognosis

Clinicopathological Features and Survival of Signet-Ring Cell Carcinoma and Mucinous Adenocarcinoma of Right Colon, Left Colon, and Rectum.
Zhu L, Ling C, Xu T, Zhang J, Zhang Y, Liu Y, Fang C, Yang L, Zhuang W, Wang R, Ping J, Wang M
Pathol Oncol Res 2021;27:1609800. Epub 2021 Jul 2 doi: 10.3389/pore.2021.1609800. PMID: 34276258Free PMC Article
Clinical Analysis of Primary Colorectal Signet-Ring Cell Carcinoma.
Liang Z, Yan D, Li G, Cheng H
Clin Colorectal Cancer 2018 Mar;17(1):e39-e44. Epub 2017 Jul 8 doi: 10.1016/j.clcc.2017.06.010. PMID: 28789931
Is signet-ring cell carcinoma a specific entity among gastric cancers?
Voron T, Messager M, Duhamel A, Lefevre J, Mabrut JY, Goere D, Meunier B, Brigand C, Hamy A, Glehen O, Mariette C, Paye F
Gastric Cancer 2016 Oct;19(4):1027-1040. Epub 2015 Nov 25 doi: 10.1007/s10120-015-0564-2. PMID: 26606931
Signet-ring cell carcinoma of the stomach: Impact on prognosis and specific therapeutic challenge.
Pernot S, Voron T, Perkins G, Lagorce-Pages C, Berger A, Taieb J
World J Gastroenterol 2015 Oct 28;21(40):11428-38. doi: 10.3748/wjg.v21.i40.11428. PMID: 26523107Free PMC Article
Primary signet ring cell carcinoma of the colon and rectum.
Arifi S, Elmesbahi O, Amarti Riffi A
Bull Cancer 2015 Oct;102(10):880-8. Epub 2015 Sep 26 doi: 10.1016/j.bulcan.2015.07.005. PMID: 26412710

Clinical prediction guides

Single-Cell Profiling of Tumor Immune Microenvironment Reveals Immune Irresponsiveness in Gastric Signet-Ring Cell Carcinoma.
Chen J, Liu K, Luo Y, Kang M, Wang J, Chen G, Qi J, Wu W, Wang B, Han Y, Shi L, Wang K, Han X, Ma X, Liu W, Ding Y, Wang L, Liang H, Wang L, Chen J
Gastroenterology 2023 Jul;165(1):88-103. Epub 2023 Mar 14 doi: 10.1053/j.gastro.2023.03.008. PMID: 36921674
Clinicopathological and Molecular Characteristics of Colorectal Signet Ring Cell Carcinoma: A Review.
An Y, Zhou J, Lin G, Wu H, Cong L, Li Y, Qiu X, Shi W
Pathol Oncol Res 2021;27:1609859. Epub 2021 Jul 26 doi: 10.3389/pore.2021.1609859. PMID: 34381313Free PMC Article
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Gastric adenocarcinoma of fundic gland type with signet-ring cell carcinoma component: A case report and review of the literature.
Kai K, Satake M, Tokunaga O
World J Gastroenterol 2018 Jul 14;24(26):2915-2920. doi: 10.3748/wjg.v24.i26.2915. PMID: 30018486Free PMC Article
Signet-ring cell carcinoma of the stomach: Impact on prognosis and specific therapeutic challenge.
Pernot S, Voron T, Perkins G, Lagorce-Pages C, Berger A, Taieb J
World J Gastroenterol 2015 Oct 28;21(40):11428-38. doi: 10.3748/wjg.v21.i40.11428. PMID: 26523107Free PMC Article

Recent systematic reviews

Clinicopathological Features and Prognostic-Related Risk Factors of Gastric Signet Ring Cell Carcinoma: A Meta-Analysis.
Guo Y, Wang Q, Tian Q, Bo C, Li N, Zhang S, Li P
Comput Math Methods Med 2022;2022:3473445. Epub 2022 Aug 5 doi: 10.1155/2022/3473445. PMID: 36035278Free PMC Article
The Molecular Associations of Signet-Ring Cell Carcinoma in Colorectum: Meta-Analysis and System Review.
Liu X, Huang L, Liu M, Wang Z
Medicina (Kaunas) 2022 Jun 21;58(7) doi: 10.3390/medicina58070836. PMID: 35888555Free PMC Article
Overall Survival in Phase 3 Clinical Trials and the Surveillance, Epidemiology, and End Results Database in Patients With Metastatic Colorectal Cancer, 1986-2016: A Systematic Review.
Shen C, Tannenbaum D, Horn R, Rogers J, Eng C, Zhou S, Johnson B, Kopetz S, Morris V, Overman M, Parseghian C, Chang GJ, Lopez-Olivo MA, Kanwal R, Ellis LM, Dasari A
JAMA Netw Open 2022 May 2;5(5):e2213588. doi: 10.1001/jamanetworkopen.2022.13588. PMID: 35608860Free PMC Article
Survival Outcomes and Clinicopathological Features in Inflammatory Bowel Disease-associated Colorectal Cancer: A Systematic Review and Meta-analysis.
Lu C, Schardey J, Zhang T, Crispin A, Wirth U, Karcz KW, Bazhin AV, Andrassy J, Werner J, Kühn F
Ann Surg 2022 Nov 1;276(5):e319-e330. Epub 2021 Dec 14 doi: 10.1097/SLA.0000000000005339. PMID: 34913897
Histological and mutational profile of diffuse gastric cancer: current knowledge and future challenges.
Garcia-Pelaez J, Barbosa-Matos R, Gullo I, Carneiro F, Oliveira C
Mol Oncol 2021 Nov;15(11):2841-2867. Epub 2021 May 2 doi: 10.1002/1878-0261.12948. PMID: 33724653Free PMC Article

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Gastric Cancer, 2023

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