Loading
Rare tumours of the appendix that range from slow mucin-producing growths that fill the abdomen (pseudomyxoma peritonei) to aggressive adenocarcinomas. The slow forms are treated by extensive surgery with heated chemotherapy in the abdomen; the fast ones like colon cancer.
Appendiceal neoplasms include low-grade appendiceal mucinous neoplasms (LAMN) that rupture and seed the peritoneum as pseudomyxoma peritonei (PMP), mucinous and non-mucinous adenocarcinomas, goblet cell adenocarcinoma, and neuroendocrine tumours (the most common appendix tumour, usually cured by appendicectomy). PSOGI grading (acellular mucin, low-grade, high-grade, signet ring) predicts outcome. GNAS mutations mark low-grade mucinous disease; KRAS, TP53 and SMAD4 mark higher grade.
For PMP and peritoneal disease, cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC, usually mitomycin C) at an experienced centre gives 10-year survival of ~60-70% for low-grade disease (Sugarbaker, Chua 2012). Systemic chemotherapy (FOLFOX/CAPOX) is used for high-grade and unresectable disease but a randomised trial (2024) showed no benefit in low-grade mucinous carcinoma peritonei. Iterative CRS, mucolytics (bromelain-acetylcysteine) and intraperitoneal therapies are under study. Appendiceal adenocarcinoma is otherwise managed like colorectal cancer, with right hemicolectomy and stage-based chemotherapy.
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.
About 1-2 per 100,000 per year and rising, especially in adults under 50; most are found incidentally at appendicectomy.
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), 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
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Appendicectomy (LAMN without perforation) or right hemicolectomy (adenocarcinoma, goblet cell); adjuvant chemotherapy for node-positive adenocarcinoma by colorectal analogy.
Cytoreductive surgery with HIPEC at a peritoneal-surface-malignancy centre; repeat CRS for recurrence when feasible.
FOLFOX/CAPOX ± bevacizumab; systemic chemotherapy has no proven benefit in low-grade disease (randomised 2024).
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"Appendiceal cancer and pseudomyxoma peritonei" OR ABSTRACT:"Appendiceal cancer and pseudomyxoma peritonei" OR TITLE:"Appendix cancer" OR ABSTRACT:"Appendix cancer" OR TITLE:"PMP" OR ABSTRACT:"PMP" OR TITLE:"Low-grade appendiceal mucinous neoplasm LAMN" OR ABSTRACT:"Low-grade appendiceal mucinous neoplasm LAMN") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Appendiceal cancer and pseudomyxoma peritonei, 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.
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.
Death of brain tissue months to years after radiosurgery or high-dose brain radiotherapy, which can look exactly like tumour growing back on a scan.
Blood clots in the leg veins or lungs. Cancer makes blood clot more easily and some treatments (IMiDs, anti-VEGF drugs, hormone therapy, central lines, surgery) add risk; clots are the second commonest cause of death in cancer patients after the cancer itself.
See all on the product pages:BevacizumabCAPOX (capecitabine, oxaliplatin)FOLFOX (5-FU, leucovorin, oxaliplatin)·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Appendiceal cancer and pseudomyxoma peritonei, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.