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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.
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).
How 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.
How common: about 1 percent (Discover Oncology 2021).
Treatment: 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.
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).
Right-sided tumours behave differently from left-sided and rectal ones; the colon drains along its mesenteric vessels, the rectum into the mesorectum and pelvic side wall.
Same organ: Colon cancer (adenocarcinoma of the colon), Mucinous adenocarcinoma of the colon and rectum, Medullary carcinoma of the colon, Serrated adenocarcinoma of the colon and rectum, Peritoneal mesothelioma, Colorectal cancer, Rectal cancer, Mismatch-repair deficient (MSI-high) colorectal cancer, BRAF V600E-mutant colorectal cancer, HER2-amplified colorectal cancer, KRAS G12C-mutant colorectal cancer, Early-onset colorectal cancer (under 50), Anal cancer (squamous cell carcinoma), Appendiceal cancer and pseudomyxoma peritonei, Small intestine cancer (small bowel adenocarcinoma), Small intestinal neuroendocrine tumours, Anal high-grade squamous intraepithelial lesions (precursor), Localised anal squamous cell carcinoma (stage I to III), Metastatic and recurrent anal squamous cell carcinoma, Low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei, Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell), Goblet cell adenocarcinoma of the appendix, Localised small bowel adenocarcinoma (stage I to III, resected), Advanced and metastatic small bowel adenocarcinoma
Treated as the colorectal page by stage and mismatch repair status; no trial in the type.
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Query for this cancer: (TITLE:"Signet ring cell carcinoma of the colon and rectum" OR ABSTRACT:"Signet ring cell carcinoma of the colon and rectum" OR TITLE:"Signet-ring cell colorectal cancer" OR ABSTRACT:"Signet-ring cell colorectal cancer" OR TITLE:"Signet ring cell carcinoma" OR ABSTRACT:"Signet ring cell carcinoma" OR TITLE:"Signet ring cell adenocarcinoma of the colon" OR ABSTRACT:"Signet ring cell adenocarcinoma of the colon" OR TITLE:"Signet-ring cell carcinoma of the colorectum" OR ABSTRACT:"Signet-ring cell carcinoma of the colorectum") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Signet ring cell carcinoma of the colon and rectum, not a curated reading list.
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