{"entity":{"id":"colorectal-signet-ring-cell-carcinoma","kind":"cancer","name":"Signet ring cell carcinoma of the colon and rectum","aka":["Signet-ring cell colorectal cancer","Signet ring cell carcinoma","Signet ring cell adenocarcinoma of the colon","Signet-ring cell carcinoma of the colorectum"],"tldr":"Signet ring cell carcinoma is a rare form of bowel cancer, about one in a hundred, in which the cells fill with mucus that pushes the nucleus aside, like a ring. It occurs in younger people, favours the right colon, is usually found at an advanced stage and often spreads across the lining of the abdomen, so its outlook is poor; it is treated like other bowel cancer for want of trials of its own.","summary":"The WHO classification defines signet ring cell carcinoma by signet ring cells making up more than 50 percent of the tumour (Nagtegaal 2020). The review evidence associates it with young age, proximal site, advanced stage, high grade, frequent node involvement, peritoneal metastasis and a significantly poor prognosis; molecularly, the classic WNT, RAS-MAPK and PI3K pathways are mutated less often than in ordinary adenocarcinoma while RNF43, CDH1, SMAD4 and TGF-beta pathway genes are altered more often, and many but not all studies report more BRAF mutation and microsatellite instability (Pathology Oncology Research 2021). In the meta-analysis, signet ring cell patients were younger and twice as likely to have right-sided disease (odds ratio 2.12), and five-year overall survival was worse than adenocarcinoma (hazard ratio 2.54 for signet ring and mucinous combined) (Discover Oncology 2021). In the Munich registry it was diagnosed at more advanced stages, with reduced survival (J Cancer Res Clin Oncol 2016).\n\nHow it differs from its parent: it is the histology with the worst outlook among colorectal cancers and with peritoneal rather than liver spread, and it shares its cell type and CDH1 biology with diffuse gastric cancer rather than with ordinary colon adenocarcinoma.\n\nHow common: about 1 percent (Discover Oncology 2021).\n\nTreatment: as the parent by stage and mismatch repair status; peritoneal disease follows the colorectal page's approach to peritoneal metastases; there is no trial in the type.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Signet_ring_cell_carcinoma","links":[{"label":"Nagtegaal 2020, Histopathology: the 2019 WHO classification of tumours of the digestive system","url":"https://doi.org/10.1111/his.13975"},{"label":"Pathology Oncology Research 2021: clinicopathological and molecular characteristics of colorectal signet ring cell carcinoma (review)","url":"https://doi.org/10.3389/pore.2021.1609859"},{"label":"Discover Oncology 2021: signet ring cell and mucinous carcinoma versus adenocarcinoma, meta-analysis of 1,087,055 patients","url":"https://doi.org/10.1007/s12672-021-00398-6"},{"label":"J Cancer Res Clin Oncol 2016: mucinous and signet ring cell colorectal cancer in 28,056 Munich registry patients","url":"https://doi.org/10.1007/s00432-016-2224-2"}],"tags":["subtype-page","wave4","colorectal"],"related":["colorectal","colon-cancer","colorectal-mucinous-adenocarcinoma","early-onset-colorectal","appendiceal-adenocarcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["folfox"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"About 1 percent of colorectal cancers: 11,510 of 1,087,055 patients (1.06 percent) in the meta-analysis (Discover Oncology 2021) and 160 of 28,056 (0.6 percent) in the Munich registry (J Cancer Res Clin Oncol 2016).","subtypes":["Signet ring cell carcinoma, mismatch-repair deficient (MSI-high; right-sided)","Signet ring cell carcinoma, mismatch-repair proficient (CDH1, RNF43 and SMAD4 altered; peritoneal spread)","Mucinous adenocarcinoma with signet ring cells under 50 percent (classified as mucinous)"],"biomarkers":["Signet ring cells over 50 percent of the tumour","Mismatch repair status","CDH1, RNF43 and SMAD4 alterations (research)","Peritoneal disease on imaging"],"standardOfCare":[{"setting":"All stages","approach":"Treated as the colorectal page by stage and mismatch repair status; no trial in the type.","refs":["colorectal","colon-cancer","folfox"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"colorectal"},"route":"/cancers/colorectal-signet-ring-cell-carcinoma/","neighbours":{"cancer":[{"id":"appendiceal-adenocarcinoma","kind":"cancer","name":"Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell)","route":"/cancers/appendiceal-adenocarcinoma/"},{"id":"colon-cancer","kind":"cancer","name":"Colon cancer (adenocarcinoma of the colon)","route":"/cancers/colon-cancer/"},{"id":"colorectal","kind":"cancer","name":"Colorectal cancer","route":"/cancers/colorectal/"},{"id":"early-onset-colorectal","kind":"cancer","name":"Early-onset colorectal cancer (under 50)","route":"/cancers/early-onset-colorectal/"},{"id":"colorectal-mucinous-adenocarcinoma","kind":"cancer","name":"Mucinous adenocarcinoma of the colon and rectum","route":"/cancers/colorectal-mucinous-adenocarcinoma/"}],"drug":[{"id":"folfox","kind":"drug","name":"FOLFOX (5-FU, leucovorin, oxaliplatin)","route":"/drugs/folfox/"}]}}