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Appendix Signet Ring Cell Carcinoma

MedGen UID:
328324
•Concept ID:
C1711320
•
Neoplastic Process
Synonym: Appendix Signet-Ring Cell Carcinoma

Definition

An adenocarcinoma arising from the appendix, characterized by the presence of signet-ring, mucin-producing malignant cells. The signet-ring cells constitute more than fifty-percent of the malignant cells. [from NCI]

Term Hierarchy

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

Recent clinical studies

Etiology

Impact of signet ring cells on overall survival in peritoneal disseminated appendix cancer treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Shamavonian R, Lansom JD, Karpes JB, Alzahrani NA, Morris DL
Eur J Surg Oncol 2021 Jan;47(1):194-198. Epub 2020 Nov 28 doi: 10.1016/j.ejso.2020.11.134. PMID: 33272738
An Unusual Progression of Signet-Ring Cell Carcinoma of the Appendix in a Caucasian Woman.
Davis K, Bellizzi AM, Chan CHF
J Gastrointest Cancer 2019 Jun;50(2):331-333. doi: 10.1007/s12029-017-0018-7. PMID: 29043541
Evaluation of appendiceal mucinous neoplasms with a new classification system and literature review.
Gündoğar Ö, Kımıloğlu E, Komut N, Cin M, Bektaş S, Gönüllü D, İlgün AS, Erdoğan N
Turk J Gastroenterol 2018 Sep;29(5):533-542. doi: 10.5152/tjg.2018.17605. PMID: 30260774Free PMC Article
Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix.
Lieu CH, Lambert LA, Wolff RA, Eng C, Zhang N, Wen S, Rafeeq S, Taggart M, Fournier K, Royal R, Mansfield P, Overman MJ
Ann Oncol 2012 Mar;23(3):652-658. Epub 2011 Jun 8 doi: 10.1093/annonc/mdr279. PMID: 21653683Free PMC Article
Krukenberg tumor of the ovary from an occult appendiceal primary: case report and literature review.
Thomas R, Barnhill D, Worsham F, Hoskins W
Obstet Gynecol 1985 Mar;65(3 Suppl):95S-98S. PMID: 2983277

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
Imaging in secondary tumors of the ovary.
Karaosmanoglu AD, Onur MR, Salman MC, Usubutun A, Karcaaltincaba M, Ozmen MN, Akata D
Abdom Radiol (NY) 2019 Apr;44(4):1493-1505. doi: 10.1007/s00261-018-1809-4. PMID: 30361868
An Unusual Progression of Signet-Ring Cell Carcinoma of the Appendix in a Caucasian Woman.
Davis K, Bellizzi AM, Chan CHF
J Gastrointest Cancer 2019 Jun;50(2):331-333. doi: 10.1007/s12029-017-0018-7. PMID: 29043541
Education and Imaging. Gastrointestinal: mucinous and signet cell adenocarcinoma of the appendix.
Hashash JG, Nasr JY, Francis F
J Gastroenterol Hepatol 2013 Jun;28(6):903. doi: 10.1111/jgh.12226. PMID: 23692577
Primary appendiceal adenocarcinoma.
Ozakyol AH, Sariçam T, Kabukçuoğlu S, Cağa T, Erenoğlu E
Am J Clin Oncol 1999 Oct;22(5):458-9. doi: 10.1097/00000421-199910000-00007. PMID: 10521058

Therapy

Clinical benefits of FOLFOXIRI combined with bevacizumab for advanced-stage primary signet ring cell carcinoma of the appendix: A case report.
Huang N, Lu Y, Wang R, Gao P, Liu G
Medicine (Baltimore) 2023 Aug 4;102(31):e34412. doi: 10.1097/MD.0000000000034412. PMID: 37543827Free PMC Article
Clinics and pathology of Krukenberg tumor: a systematic review and meta-analysis.
Lionetti R, DE Luca M, Raffone A, Travaglino A, Coppellotti A, Peltrini R, Bracale U, D'Ambra M, Insabato L, Zullo F, D'Armiento M, Corcione F
Minerva Obstet Gynecol 2022 Aug;74(4):356-363. Epub 2021 May 4 doi: 10.23736/S2724-606X.21.04797-7. PMID: 33944524
Outcomes in Peritoneal Dissemination from Signet Ring Cell Carcinoma of the Appendix Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy.
Munoz-Zuluaga C, Sardi A, King MC, Nieroda C, Sittig M, MacDonald R, Gushchin V
Ann Surg Oncol 2019 Feb;26(2):473-481. Epub 2018 Dec 6 doi: 10.1245/s10434-018-7007-3. PMID: 30523470
Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix.
Lieu CH, Lambert LA, Wolff RA, Eng C, Zhang N, Wen S, Rafeeq S, Taggart M, Fournier K, Royal R, Mansfield P, Overman MJ
Ann Oncol 2012 Mar;23(3):652-658. Epub 2011 Jun 8 doi: 10.1093/annonc/mdr279. PMID: 21653683Free PMC Article
Primary signet ring cell carcinoma of the appendix: a rare case report and our 18-year experience.
Ko YH, Jung CK, Oh SN, Kim TH, Won HS, Kang JH, Kim HJ, Kang WK, Oh ST, Hong YS
World J Gastroenterol 2008 Oct 7;14(37):5763-8. doi: 10.3748/wjg.14.5763. PMID: 18837098Free PMC Article

Prognosis

Targeted Next-Generation Sequencing Improves the Prognostication of Patients with Disseminated Appendiceal Mucinous Neoplasms (Pseudomyxoma Peritonei).
Wald AI, Pingpank JF, Ongchin M, Hall LB, Jones H, Altpeter S, Liebdzinski M, Hamed AB, Derby J, Nikiforova MN, Bell PD, Paniccia A, Zureikat AH, Gorantla VC, Rhee JC, Thomas R, Bartlett DL, Smith K, Henn P, Theisen BK, Shyu S, Shalaby A, Choudry MHA, Singhi AD
Ann Surg Oncol 2023 Nov;30(12):7517-7526. Epub 2023 Jun 14 doi: 10.1245/s10434-023-13721-y. PMID: 37314541
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
Imaging in secondary tumors of the ovary.
Karaosmanoglu AD, Onur MR, Salman MC, Usubutun A, Karcaaltincaba M, Ozmen MN, Akata D
Abdom Radiol (NY) 2019 Apr;44(4):1493-1505. doi: 10.1007/s00261-018-1809-4. PMID: 30361868
An Unusual Progression of Signet-Ring Cell Carcinoma of the Appendix in a Caucasian Woman.
Davis K, Bellizzi AM, Chan CHF
J Gastrointest Cancer 2019 Jun;50(2):331-333. doi: 10.1007/s12029-017-0018-7. PMID: 29043541
Primary appendiceal adenocarcinoma.
Ozakyol AH, Sariçam T, Kabukçuoğlu S, Cağa T, Erenoğlu E
Am J Clin Oncol 1999 Oct;22(5):458-9. doi: 10.1097/00000421-199910000-00007. PMID: 10521058

Clinical prediction guides

Morphologic Heterogeneity of Carcinoma with Signet Ring Cell Features at Different Primary Sites.
Dahoud W, Gullo I, Imam R, Tang LH
Pathobiology 2024;91(4):279-287. Epub 2023 Dec 23 doi: 10.1159/000535941. PMID: 38142679
Targeted Next-Generation Sequencing Improves the Prognostication of Patients with Disseminated Appendiceal Mucinous Neoplasms (Pseudomyxoma Peritonei).
Wald AI, Pingpank JF, Ongchin M, Hall LB, Jones H, Altpeter S, Liebdzinski M, Hamed AB, Derby J, Nikiforova MN, Bell PD, Paniccia A, Zureikat AH, Gorantla VC, Rhee JC, Thomas R, Bartlett DL, Smith K, Henn P, Theisen BK, Shyu S, Shalaby A, Choudry MHA, Singhi AD
Ann Surg Oncol 2023 Nov;30(12):7517-7526. Epub 2023 Jun 14 doi: 10.1245/s10434-023-13721-y. PMID: 37314541
Impact of signet ring cells on overall survival in peritoneal disseminated appendix cancer treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
Shamavonian R, Lansom JD, Karpes JB, Alzahrani NA, Morris DL
Eur J Surg Oncol 2021 Jan;47(1):194-198. Epub 2020 Nov 28 doi: 10.1016/j.ejso.2020.11.134. PMID: 33272738
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
Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix.
Lieu CH, Lambert LA, Wolff RA, Eng C, Zhang N, Wen S, Rafeeq S, Taggart M, Fournier K, Royal R, Mansfield P, Overman MJ
Ann Oncol 2012 Mar;23(3):652-658. Epub 2011 Jun 8 doi: 10.1093/annonc/mdr279. PMID: 21653683Free PMC Article

Recent systematic reviews

Clinics and pathology of Krukenberg tumor: a systematic review and meta-analysis.
Lionetti R, DE Luca M, Raffone A, Travaglino A, Coppellotti A, Peltrini R, Bracale U, D'Ambra M, Insabato L, Zullo F, D'Armiento M, Corcione F
Minerva Obstet Gynecol 2022 Aug;74(4):356-363. Epub 2021 May 4 doi: 10.23736/S2724-606X.21.04797-7. PMID: 33944524

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