The first 60 days: Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell)

What happens now

Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.

Biomarkers your report may list

Histological subtype and grade (mucinous, non-mucinous, signet ring)TNM stage after right hemicolectomy Peritoneal cancer index and completeness of cytoreduction KRAS, GNAS, TP53 and SMAD4 mutations Mismatch repair and microsatellite status (uncommon deficiency)CEA, CA 19-9 and CA-125

Ask which of these were tested and what the results were; see the report reader for what each value means.

Tests and imaging linked to this cancer

CT (computed tomography)MRI

Weeks 1 to 3

Who is on your team

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These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.

A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.

Weeks 2 to 6

Decisions coming up

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Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.

1.Localised disease NCCN Guidelines: Colon Cancer (appendiceal section)
Right hemicolectomy with lymphadenectomy; adjuvant FOLFOX or CAPOX for node-positive or high-risk stage II disease, extrapolated from colon cancer.
Colectomy Lymphadenectomy (lymph node dissection)FOLFOX (5-FU, leucovorin, oxaliplatin)CAPOX (capecitabine, oxaliplatin)
2.Peritoneal metastases, resectable NCCN Guidelines: Colon Cancer (appendiceal section)
Cytoreductive surgery with HIPEC (mitomycin or oxaliplatin) in fit patients with limited disease and favourable histology, with perioperative systemic chemotherapy.
HIPEC / PIPAC (intraperitoneal chemotherapy)HIPEC (hyperthermic intraperitoneal chemotherapy)Mitomycin C FOLFOX (5-FU, leucovorin, oxaliplatin)CAPOX (capecitabine, oxaliplatin)Peritoneal metastasis
3.Unresectable or distant metastatic disease NCCN Guidelines: Colon Cancer (appendiceal section)
FOLFOX or CAPOX with or without bevacizumab; FOLFIRI in second line; pembrolizumab for mismatch-repair-deficient tumours.
FOLFOX (5-FU, leucovorin, oxaliplatin)CAPOX (capecitabine, oxaliplatin)Bevacizumab FOLFIRI (5-FU, leucovorin, irinotecan)Pembrolizumab Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR)
4.Signet ring cell carcinoma with extensive peritoneal disease NCCN Guidelines: Colon Cancer (appendiceal section)
Systemic chemotherapy first; cytoreduction only for exceptional responders.
FOLFOX (5-FU, leucovorin, oxaliplatin)CAPOX (capecitabine, oxaliplatin)HIPEC / PIPAC (intraperitoneal chemotherapy)

Every visit

Questions to ask at each visit

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Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.

Newly diagnosed

What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
Which biomarkers have been tested on my tumour (for example Histological subtype and grade, TNM stage after right hemicolectomy, Peritoneal cancer index and completeness of cytoreduction, KRAS, GNAS, TP53 and SMAD4 mutations, Mismatch repair and microsatellite status), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Mucinous appendiceal adenocarcinoma, Non-mucinousappendiceal adenocarcinoma, Signet ring cell carcinoma of the appendix.
Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.

Localised disease

For my situation (localised disease), which of the standard options do you recommend and why?Guideline options include: Right hemicolectomy with lymphadenectomy; adjuvant FOLFOX or CAPOX for node-positive or high-risk stage II disease, extrapolated from colon cancer.
Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.

Peritoneal metastases, resectable

For my situation (peritoneal metastases, resectable), which of the standard options do you recommend and why?Guideline options include: Cytoreductive surgery with HIPEC (mitomycin or oxaliplatin) in fit patients with limited disease and favourable histology, with perioperative systemic chemotherapy.
Am I a candidate for Mitomycin C, FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.

Unresectable or distant metastatic disease

For my situation (unresectable or distant metastatic disease), which of the standard options do you recommend and why?Guideline options include: FOLFOX or CAPOX with or without bevacizumab; FOLFIRI in second line; pembrolizumab for mismatch-repair-deficient tumours.
Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), Bevacizumab or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.

Signet ring cell carcinoma with extensive peritoneal disease

For my situation (signet ring cell carcinoma with extensive peritoneal disease), which of the standard options do you recommend and why?Guideline options include: Systemic chemotherapy first; cytoreduction only for exceptional responders.
Am I a candidate for FOLFOX (5-FU, leucovorin, oxaliplatin), CAPOX (capecitabine, oxaliplatin), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

Are there clinical trials I could join, for example of FOLFOX (5-FU, leucovorin, oxaliplatin), HIPEC / PIPAC (intraperitoneal chemotherapy), Pembrolizumab?Trials are how the next standard of care is set; asking early keeps options open.
Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
I read that “No adjuvant chemotherapy trial has ever been run in appendiceal adenocarcinoma”. How does that affect my plan?Open problems are where trials and second opinions matter most.
I read that “Which patients with peritoneal disease benefit from HIPEC is defined only by retrospective series”. How does that affect my plan?Open problems are where trials and second opinions matter most.

From day one

Help that costs nothing

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Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.

Any time

What to read next

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Words this page and the cancer page use

Peritoneal metastases from bowel cancer: Bowel cancer can seed the lining of the abdomen rather than travel to the liver or lungs.
Peritoneal cancer index: The peritoneal cancer index is the score a surgeon gives the abdomen at operation, region by region, for how much tumour is on its lining.
Cytoreductive surgery: Cytoreductive surgery is an operation that strips every visible tumour deposit from the lining of the abdomen and from the organs it coats, often taking many hours.
HIPEC (hyperthermic intraperitoneal chemotherapy): After surgeons remove all visible tumour from the abdominal lining, the abdomen is bathed for 60-90 minutes in heated chemotherapy to kill the microscopic cells left behind.
Colectomy: Removing the part of the colon containing the cancer along with its blood supply and lymph nodes, then joining the ends.
Peritoneal metastasis: Spread across the lining of the abdomen, the most common way stomach cancer recurs and the hardest to treat.
Lymphadenectomy (lymph node dissection): Surgically removing the lymph nodes that drain a tumour, both to stage the cancer and to clear any spread.
Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR): Microsatellite instability is the mark of a broken DNA spell-checker (loss of MLH1, MSH2, MSH6 or PMS2) that leaves thousands of mutations, so the tumour displays abnormal proteins that T cells can recognise.

Every term links to the glossary.