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Appendiceal adenocarcinoma is the invasive, gland-forming form of appendix cancer that behaves more like bowel cancer than the jelly-producing low-grade tumours, spreading to lymph nodes and the abdominal lining. It is treated with right hemicolectomy and bowel-cancer chemotherapy, and peritoneal spread with cytoreductive surgery and heated intraperitoneal chemotherapy in fit patients.
Appendiceal adenocarcinoma invades the wall of the appendix as a carcinoma and is subdivided into mucinous adenocarcinoma (more than half the tumour is extracellular mucin), non-mucinous or colonic-type adenocarcinoma, and signet ring cell carcinoma when half or more of the cells are signet ring cells. Most are found after appendicectomy for suspected appendicitis or at operation for peritoneal disease, and staging follows the colorectal TNM system. The genetics differ from colorectal cancer: KRAS and GNAS mutations are common, APC mutations rare, microsatellite instability uncommon, and TP53 and SMAD4 mutations mark high-grade tumours; a 2025 analysis of appendiceal cancers also found that many were diagnosed in people under 50, mirroring early-onset colorectal cancer.
Because the disease is rare, treatment is extrapolated from colon cancer. Right hemicolectomy with lymphadenectomy is recommended for all adenocarcinomas, and adjuvant FOLFOX or CAPOX for node-positive or high-risk disease, without trial evidence specific to the appendix. Peritoneal metastases, the dominant pattern of spread, are treated with cytoreductive surgery and HIPEC in patients with a limited peritoneal cancer index and non-signet-ring histology, where series report median survival of several years, and with perioperative systemic chemotherapy; signet ring cell carcinoma with a high peritoneal cancer index does poorly even after cytoreduction. Unresectable or distant metastatic disease receives FOLFOX or CAPOX with or without bevacizumab, then FOLFIRI, and the rare mismatch-repair-deficient tumour is a candidate for pembrolizumab. Retrospective data suggest that non-mucinous tumours respond to chemotherapy like colorectal cancer while mucinous tumours respond less, and prospective trials in appendiceal cancer specifically are only now beginning.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
A minority of appendiceal tumours but the group that behaves like a true carcinoma, spreading to lymph nodes and the peritoneum; signet ring cell histology carries the worst outlook.
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), Micropapillary adenocarcinoma of the colon and rectum, Adenoma-like adenocarcinoma of the colon and rectum, Lynch syndrome-associated colorectal cancer, Familial adenomatous polyposis-associated colorectal cancer, Mucinous adenocarcinoma of the colon and rectum, Signet ring cell carcinoma 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, Goblet cell adenocarcinoma of the appendix, Localised small bowel adenocarcinoma (stage I to III, resected), Advanced and metastatic small bowel adenocarcinoma
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Right hemicolectomy with lymphadenectomy; adjuvant FOLFOX or CAPOX for node-positive or high-risk stage II disease, extrapolated from colon cancer.
Cytoreductive surgery with HIPEC (mitomycin or oxaliplatin) in fit patients with limited disease and favourable histology, with perioperative systemic chemotherapy.
FOLFOX or CAPOX with or without bevacizumab; FOLFIRI in second line; pembrolizumab for mismatch-repair-deficient tumours.
Systemic chemotherapy first; cytoreduction only for exceptional responders.
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The names on an appendix or small bowel pathology report, and hence which OnCo subtype page applies, follow this classification.
The standard-of-care rows on the appendiceal pages, particularly who is referred for cytoreductive surgery and who receives chemotherapy, follow this consensus and the NCCN appendiceal section.
Whether an appendiceal tumour is called LAMN or adenocarcinoma, and whether peritoneal disease is graded low or high, decides prognosis and treatment; this paper set those names.
Query for this cancer: (TITLE:"Appendiceal adenocarcinoma" OR ABSTRACT:"Appendiceal adenocarcinoma" OR TITLE:"mucinous and non-mucinous, including signet ring cell" OR ABSTRACT:"mucinous and non-mucinous, including signet ring cell" OR TITLE:"Appendix adenocarcinoma" OR ABSTRACT:"Appendix adenocarcinoma" OR TITLE:"Colonic-type appendiceal adenocarcinoma" OR ABSTRACT:"Colonic-type appendiceal adenocarcinoma" OR TITLE:"Mucinous appendiceal adenocarcinoma" OR ABSTRACT:"Mucinous appendiceal adenocarcinoma" OR TITLE:"Signet ring cell carcinoma of the appendix" OR ABSTRACT:"Signet ring cell carcinoma of the appendix") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell), not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
The bowel cancer regimens share low blood counts, tiredness, sickness and a sore mouth. Oxaliplatin adds cold-triggered tingling, irinotecan adds early and late diarrhoea, capecitabine adds hand-foot syndrome and needs a DPD test first, and cetuximab or panitumumab add an acne-like rash and low magnesium. The rule for all of them: ring the 24-hour number rather than wait.
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
See all on the product pages:BevacizumabCAPOX (capecitabine, oxaliplatin)FOLFIRI (5-FU, leucovorin, irinotecan)FOLFOX (5-FU, leucovorin, oxaliplatin)Mitomycin CPembrolizumab·Printable cards in the navigator
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