Peritoneal mesothelioma
Peritoneal mesothelioma grows in the lining of the abdomen, causing swelling, pain and fluid. Unlike its pleural cousin it is often treated with major surgery to strip the lining followed by heated chemotherapy washed through the abdomen, which can give long survival in fit patients with epithelioid disease.
Overview
Peritoneal mesothelioma presents with abdominal distension from ascites, pain, weight loss or a mass found at surgery, and is diagnosed by laparoscopic biopsy with the same immunohistochemistry as pleural disease. Asbestos exposure is found in only a minority; BAP1 germline mutations account for some cases, and the disease is commoner in women than pleural mesothelioma. For fit patients with epithelioid histology and disease that can be removed, cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) is the standard in specialist centres and gives median survival of five years or more in series, though no randomised trial exists. Patients who are not candidates receive platinum-pemetrexed chemotherapy, with immunotherapy increasingly used on the basis of small trials and the pleural data. Well-differentiated papillary and multicystic forms behave almost benignly and are managed separately.
State of the art
- Immunotherapy is being adopted from pleural mesothelioma with encouraging early results, and peritoneal patients are now included in mesothelin-directed trials.
- Registries and consortia have replaced case series as the evidence base because randomised trials are so hard to run in a disease this rare.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- Cytoreduction with HIPEC, pioneered by Sugarbaker, turned a disease with a median survival under a year into one where a substantial fraction of selected patients live five years or more.
Anatomy and lymph node drainage
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.
- Right colon (MSI-high, BRAF commoner)
- Left colon and sigmoid
- Rectum
- Anal canal (HPV squamous)
- Appendix
- Peritoneum and omentum (mesothelioma, peritoneal spread)Well-differentiated papillary mesothelial tumour (indolent) · Multicystic mesothelioma (indolent)
- pericolic
- mesenteric root
- para-aortic
- mesorectal
- lateral pelvic and inguinal (anal)
Same organ: Colorectal cancer, Anal cancer (squamous cell carcinoma), Appendiceal cancer and pseudomyxoma peritonei, Small intestine cancer (small bowel adenocarcinoma)
About one in ten mesotheliomas; a few hundred cases a year in the US. The asbestos link is weaker than in the pleura, it affects more women and younger people, and selected patients live many years after surgery.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
CT, laparoscopy with biopsy and scoring of disease extent; germline BAP1 testing when young or with a family history.
Cytoreductive surgery with HIPEC in an experienced centre; long-term survival in a substantial fraction.
Platinum-pemetrexed chemotherapy; nivolumab plus ipilimumab or pembrolizumab combinations extrapolated from pleural trials and small peritoneal series.
Well-differentiated papillary and multicystic tumours: surgical removal and surveillance; systemic therapy rarely needed.
Subtypes & biomarkers
top- Epithelioid (most cases)
- Biphasic and sarcomatoid (rare, poor prognosis)
- Well-differentiated papillary mesothelial tumour (indolent)
- Multicystic mesothelioma (indolent)
- Histology and peritoneal cancer index at laparoscopy
- BAP1 loss (somatic and germline)
- Ki-67 (prognostic after surgery)
- Completeness of cytoreduction score
How often this target appears
- 1908Miller and Wynn describe peritoneal mesothelioma
Among the earliest descriptions of a primary tumour of the peritoneum.
- 1995Sugarbaker develops cytoreduction with HIPEC
Peritonectomy procedures and heated intraperitoneal chemotherapy applied to peritoneal surface malignancy.
- 2009Multi-institutional registry of 405 patients
Yan and colleagues report median survival of 53 months after cytoreduction and HIPEC.
- 2021Immunotherapy series in peritoneal disease
Early phase 2 and retrospective data for nivolumab plus ipilimumab and for atezolizumab plus bevacizumab.
What is in development for Peritoneal mesothelioma, drawn from the whole corpus: 1 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Ideas not yet in a trial · 1
Open problems and what is being done
No randomised trial has compared surgery plus HIPEC with systemic therapy.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Selection for surgery relies on centre experience rather than validated criteria.
Peritoneal patients were excluded from the pleural immunotherapy trials, so their benefit is inferred.
and how the field plans to fix it →What is being done about thisAdvanced and metastatic diseaseAvailable now- HIPEC / PIPAC (intraperitoneal chemotherapy)Established
- Bone-modifying agents (bisphosphonates, denosumab)Standard of care
- DordaviproneApproved
- Laser interstitial thermal therapy (LITT)Established
- Liquid biopsy (ctDNA)Standard of care
- Lutetium-177 vipivotide tetraxetanApproved
In trials- ACTIONRecruiting
- CAR-T for glioma (IL13Rα2, GD2, EGFRvIII, multi-target)Phase 1
- CIRCULATE-Japan (GALAXY / VEGA / ALTAIR)Active
- DESTINY-Breast12Positive
- DYNAMICPositive
- EF-14Positive
Ideas and roadmaps- A billion-dollar prize for the first durable cure of a lethal metastatic cancer
- A blood test for the pre-metastatic niche
- A global rapid tissue donation network for metastatic disease
- A national rapid research autopsy network for end-stage cancer
- A regulatory endpoint for drugs that block spread, not tumours
- A ring-fenced metastasis programme with metastasis-specific endpoints
Background: Blood-brain barrier (BBB), Circulating tumour DNA (ctDNA), EGFRvIII, Epithelial-mesenchymal transition & drug efflux, H3 K27M (diffuse midline glioma). Also on OnCo: Atlas of advanced disease · Invasion and metastasis.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- via Mesothelioma
- via Mesothelioma
- Johns Hopkins Hospital / Sidney Kimmel Comprehensive Cancer CenterBaltimore, USNewsweek oncology #10NCI comprehensivevia Pembrolizumab
- via Mesothelioma
- via HIPEC / PIPAC (intraperitoneal chemotherapy)
- via Mesothelioma
- via Nivolumab, Ipilimumab
- via Nivolumab, Ipilimumab
- via Nivolumab, Ipilimumab
- via Nivolumab
- Alliance for Clinical Trials in OncologyChicago, IL, USvia Pembrolizumab
- ETOP IBCSG Partners FoundationBern, CHvia Mesothelioma
- via HIPEC / PIPAC (intraperitoneal chemotherapy)
- European Society of Surgical OncologyBrussels, BEvia HIPEC / PIPAC (intraperitoneal chemotherapy)
- First Affiliated Hospital of Sun Yat-sen UniversityGuangzhou, CNvia HIPEC / PIPAC (intraperitoneal chemotherapy)
- German Hodgkin Study GroupCologne, DEvia Nivolumab
- GOG FoundationPhiladelphia, PA, USvia Pembrolizumab
- Institut Jules BordetBrussels, BEvia Pembrolizumab
- via Mesothelioma
- International Association for the Study of Lung CancerDenver, CO, USvia Mesothelioma
- Kyoto University HospitalKyoto, JPvia Nivolumab
- via Nivolumab
- via Pembrolizumab
- via Mesothelioma
- National Cancer Center Hospital EastKashiwa, Chiba, JPvia Nivolumab
- NCI Center for Cancer Research (intramural programme)Bethesda, MD, USvia Mesothelioma
- Shanghai Chest HospitalShanghai, CNvia Mesothelioma
- Society of Surgical OncologyRosemont, IL, USvia HIPEC / PIPAC (intraperitoneal chemotherapy)
- SWOG Cancer Research NetworkPortland, OR, USvia Nivolumab
- UNICANCERParis, FRvia HIPEC / PIPAC (intraperitoneal chemotherapy)
Questions to ask
topQuestions to ask your oncologist about Peritoneal mesothelioma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Histology and peritoneal cancer index at laparoscopy, BAP1 loss, Ki-67, Completeness of cytoreduction score), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Epithelioid, Biphasic and sarcomatoid, Well-differentiated papillary mesothelial tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Why: Guideline options include: CT, laparoscopy with biopsy and scoring of disease extent; germline BAP1 testing when young or with a family history.
Resectable epithelioid disease, fit patient
- For my situation (resectable epithelioid disease, fit patient), which of the standard options do you recommend and why?Why: Guideline options include: Cytoreductive surgery with HIPEC in an experienced centre; long-term survival in a substantial fraction.
Unresectable or unfit
- For my situation (unresectable or unfit), which of the standard options do you recommend and why?Why: Guideline options include: Platinum-pemetrexed chemotherapy; nivolumab plus ipilimumab or pembrolizumab combinations extrapolated from pleural trials and small peritoneal series.
- Am I a candidate for Pemetrexed, Nivolumab, Ipilimumab or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Indolent variants
- For my situation (indolent variants), which of the standard options do you recommend and why?Why: Guideline options include: Well-differentiated papillary and multicystic tumours: surgical removal and surveillance; systemic therapy rarely needed.
Any stage
- Are there clinical trials I could join, for example of Antibody-drug conjugates for mesothelioma: why they have failed so far and how they could work?Why: 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?Why: 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?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No randomised trial has compared surgery plus HIPEC with systemic therapy”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Selection for surgery relies on centre experience rather than validated criteria”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
4targets
2drugs
4companies
2ideas
1Latest papers
topQuery for this cancer: (TITLE:"Peritoneal mesothelioma" OR ABSTRACT:"Peritoneal mesothelioma" OR TITLE:"Malignant peritoneal mesothelioma" OR ABSTRACT:"Malignant peritoneal mesothelioma" OR TITLE:"MPeM" OR ABSTRACT:"MPeM" OR TITLE:"Diffuse malignant peritoneal mesothelioma" OR ABSTRACT:"Diffuse malignant peritoneal mesothelioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Peritoneal mesothelioma, not a curated reading list.
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