The first 60 days: Pleural mesothelioma

Below, week by week, is what OnCo's record of Pleural mesothelioma says about the first two months: the order is typical, the timing is yours to ask about. Pleural mesothelioma grows in the lining of the lung after asbestos exposure and causes breathlessness and chest pain from fluid and thickening. It is rarely curable; the first-line choice is nivolumab with ipilimumab or pembrolizumab with chemotherapy, and surgery to remove the lining is no longer recommended outside trials. Sections appear only where the record has something to say. Orientation, not medical advice.

Weeks 1 to 2

What happens now

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

Diagnosis and staging
CT and PET-CT; thoracoscopic biopsy with an immunohistochemistry panel; histology and BAP1/MTAP status recorded because they drive treatment.
Mesothelioma

Biomarkers your report may list

Histology (epithelioid versus non-epithelioid)BAP1 loss CDKN2A/MTAP deletion Mesothelin expression PD-L1 (not predictive for nivolumab plus ipilimumab)

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

Weeks 1 to 3

Who is on your team

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

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.Surgery
Not recommended for cure outside trials after MARS 2; talc pleurodesis or an indwelling pleural catheter controls effusion.
MARS 2
2.First line, non-epithelioid
Nivolumab plus ipilimumab (CheckMate 743).
Nivolumab Ipilimumab CheckMate 743
3.First line, epithelioid
Pembrolizumab with platinum-pemetrexed (KEYNOTE-483), nivolumab plus ipilimumab, or platinum-pemetrexed with or without bevacizumab, by fitness and preference.
Pembrolizumab Pemetrexed IND.227 / KEYNOTE-483
4.Second line
Nivolumab (CONFIRM) if not given first line; chemotherapy rechallenge or gemcitabine or vinorelbine after immunotherapy.
Nivolumab

Every visit

Questions to ask at each visit

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 Histology, BAP1 loss, CDKN2A/MTAP deletion, Mesothelin expression, PD-L1), 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 Epithelioid, Biphasic, Sarcomatoid.
Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.

Diagnosis and staging

For my situation (diagnosis and staging), which of the standard options do you recommend and why?Guideline options include: CT and PET-CT; thoracoscopic biopsy with an immunohistochemistry panel; histology and BAP1/MTAP status recorded because they drive treatment.

First line, non-epithelioid

For my situation (first line, non-epithelioid), which of the standard options do you recommend and why?Guideline options include: Nivolumab plus ipilimumab (CheckMate 743).
Am I a candidate for Nivolumab, Ipilimumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
How do the results of CheckMate 743 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.

First line, epithelioid

For my situation (first line, epithelioid), which of the standard options do you recommend and why?Guideline options include: Pembrolizumab with platinum-pemetrexed (KEYNOTE-483), nivolumab plus ipilimumab, or platinum-pemetrexed with or without bevacizumab, by fitness and preference.
Am I a candidate for Pembrolizumab, Pemetrexed, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
How do the results of IND.227 / KEYNOTE-483 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.

Surgery

For my situation (surgery), which of the standard options do you recommend and why?Guideline options include: Not recommended for cure outside trials after MARS 2; talc pleurodesis or an indwelling pleural catheter controls effusion.
How do the results of MARS 2 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.

Second line

For my situation (second line), which of the standard options do you recommend and why?Guideline options include: Nivolumab (CONFIRM) if not given first line; chemotherapy rechallenge or gemcitabine or vinorelbine after immunotherapy.
Am I a candidate for Nivolumab, 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 Antibody-drug conjugates for mesothelioma: why they have failed so far and how they could work?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 curative treatment for the majority; median survival with the best regimens is about 18 months”. How does that affect my plan?Open problems are where trials and second opinions matter most.
I read that “Sarcomatoid disease responds poorly to everything except immunotherapy”. How does that affect my plan?Open problems are where trials and second opinions matter most.

Weeks 3 to 8

Trials to ask about

Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.

From day one

Help that costs nothing

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

Words this page and the cancer page use

Asbestos: A mineral once used everywhere in building, whose fibres lodge in the lung when they are breathed in and cause cancer decades later.
Malignant pleural effusion: Fluid collecting in the space between the lung and the chest wall because cancer has reached the lining.
BAP1 loss: BAP1 is a tumour-suppressor gene that mesothelioma, uveal melanoma and clear-cell kidney cancer lose more often than any other; a lost nuclear stain on the biopsy confirms cancer over a benign look-alike in mesothelioma, marks the metastasis-prone half of eye melanomas, and, when inherited, defines a family syndrome of all three.
CDKN2A/B homozygous deletion: Losing both copies of the CDKN2A/B genes on chromosome 9p21 removes the cell's main brake on division; in an IDH-mutant glioma it alone makes the tumour grade 4, in mesothelioma and melanoma it marks aggressive disease, and the neighbouring MTAP gene usually goes with it, opening a new drug target.

Every term links to the glossary.