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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
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
Mixed Adeno-neuroendocrine Carcinoma: An Aggressive Clinical Entity.
Brathwaite S, Rock J, Yearsley MM, Bekaii-Saab T, Wei L, Frankel WL, Hays J, Wu C, Abdel-Misih S
Ann Surg Oncol 2016 Jul;23(7):2281-6. Epub 2016 Mar 10 doi: 10.1245/s10434-016-5179-2. PMID: 26965701Free 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 tumors: CT features and growth characteristics.
Kuhlman JE, Hruban RH, Fishman EK
South Med J 1989 Oct;82(10):1215-9. PMID: 2552586

Diagnosis

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
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
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
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
Prolonged Survival in Peritoneal Metastatic Appendiceal Carcinoma Patients Treated With Combined Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy.
Acs M, Gerken M, Zustin J, Blaj S, Isgandarova S, Piso P
J Surg Res 2023 Mar;283:839-852. Epub 2022 Dec 6 doi: 10.1016/j.jss.2022.10.083. PMID: 36915011
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

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
Mixed Adeno-neuroendocrine Carcinoma: An Aggressive Clinical Entity.
Brathwaite S, Rock J, Yearsley MM, Bekaii-Saab T, Wei L, Frankel WL, Hays J, Wu C, Abdel-Misih S
Ann Surg Oncol 2016 Jul;23(7):2281-6. Epub 2016 Mar 10 doi: 10.1245/s10434-016-5179-2. PMID: 26965701Free PMC Article
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
Goblet or signet ring cells: that is the question.
Wang HL, Dhall D
Adv Anat Pathol 2009 Jul;16(4):247-54. doi: 10.1097/PAP.0b013e3181a9d49a. PMID: 19546612

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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