Last modified: September 16, 2021
Mesothelioma Types by Tumor Location
Mesothelioma types differ based on the location where cancer develops and the tumor cell variant. For example, when cancer forms in the pleural lining surrounding the lungs, the cancer is called pleural mesothelioma. Mesothelioma most commonly develops on the outer surface of the lungs, but other locations include the abdomen, heart and testicles.
Peritoneal mesothelioma is the term for cancer of the abdominal lining known as the peritoneum and is the second most common variant of the disease. Two other rarer forms also exist. The rarest variant is pericardial mesothelioma, cancer of the protective heart sac. The last type is testicular mesothelioma which forms in the tunica vaginalis lining of the testes.
Each mesothelioma type can lead to different symptoms. Doctors tailor mesothelioma treatment by cell type, such as epithelioid or sarcomatoid, and cancer stage. A patient’s mesothelioma prognosis depends mainly on tumor location and the patient’s response to treatment.
Epithelioid cells are the most common type of cell found in mesothelioma tumors and are the most responsive to treatment. Other rarer cell subtypes include well-differentiated papillary cells, small cell, deciduoid, cystic, desmoplastic, adenomatoid and heterologous cells.
Pleural is the most common mesothelioma type. Approximately 70% to 75% of cases occur in the pleura, the tissue surrounding and protecting the lungs. Researchers have conducted more clinical trial studies on this mesothelioma tumor than any of the other variants.
Aggressive surgery provides the best outcome for early-stage pleural mesothelioma, but doctors often diagnose patients after symptoms appear in later stages. Typical symptoms include shortness of breath, chest pain, weight loss, fever and fatigue.
Most patients qualify for chemotherapy or radiation therapy. Based on health history, many are also eligible for clinical trials or immunotherapy. These therapies can add months or years to life expectancy. With treatment, patients can live for three or more years at stage 1 versus approximately 12 months at stage 4.
Peritoneal mesothelioma accounts for 10% to 20% of all cases. There is less research on peritoneal mesothelioma than the pleural variant; however, the prognosis for this tumor type is better. This cancer forms in the tissue that surrounds the abdominal organs.
Primary symptoms of peritoneal mesothelioma include abdominal pain and swelling, bloating, loss of appetite and bowel changes.
According to research, around half of peritoneal mesothelioma patients eligible for surgery with heated chemotherapy, a procedure known as HIPEC, live five or more years after diagnosis.
The pericardial mesothelioma tumor type is exceptionally uncommon. Around 200 cases exist in medical literature records, accounting for approximately 1% of all mesothelioma cases. With surgery, some patients live for years beyond the average six-month life expectancy.
The pericardial type develops in the protective heart sac known as the pericardium. Symptoms of this rare variant include irregular heartbeat, chest pain, difficulty breathing and cough. Of the cases on record, life expectancies range between six weeks and 15 months.
Testicular mesothelioma develops in the outer lining of the testes called the tunica vaginalis. This form of mesothelioma is the rarest. Less than 100 cases are reported in the medical literature, making up less than 1% of all mesothelioma cases.
Symptoms include scrotal swelling and painless testicular lumps. Treatment options include surgery and chemotherapy, which can help people live more than two years after diagnosis, on average.
Three Primary Mesothelioma Cell Types
The three mesothelioma cell varieties are epithelial, sarcomatoid and biphasic. Biphasic is a mix of the first two cell types. Mesothelioma doctors can tell the difference between cells based on how they look under a microscope.
An additional form of the disease includes benign mesothelioma, which is neither asbestos-related nor cancerous. Asbestos exposure causes cancer known as malignant mesothelioma.
Different mesothelioma tumors respond differently to various treatments. Epithelial or epithelioid cells respond the best, and sarcomatoid cells are more resistant to treatment. The biphasic subtype includes a mixture of both epithelial and sarcomatoid cells.
Cancer doctors take these differences into account when planning your mesothelioma treatment. The incidence of these cell types varies by cancer location.
Prevalence of Mesothelioma Tumors and Cell Types
|Cell Type||Pleural Tumors||Peritoneal Tumors|
Based on the limited number of cases reported in the medical literature, pericardial mesothelioma exhibits a roughly equal distribution of the three mesothelioma cell types.
Approximately two-thirds of testicular mesothelioma cases contain exclusively epithelial cells. The remaining third testicular cases are biphasic. Only one case of purely sarcomatoid cell disease is on record for testicular mesothelioma.
Epithelial Cell Subtype
Epithelioid mesothelioma makes up approximately 70% to 75% of all cases of asbestos-related mesothelioma cancers.
Patients with epithelioid cell tumors have the best prognosis. This subtype tends to be less aggressive and doesn’t spread as quickly as sarcomatoid and biphasic cell disease.
About 50% of pleural disease is epithelioid. Around 75% of peritoneal tumors are epithelioid.
Sarcomatoid Cell Subtype
Sarcomatoid mesothelioma is the least common mesothelioma cell category. It is the most aggressive and challenging to treat. It accounts for around 10% to 20% of all mesothelioma diagnoses.
You may hear sarcomatoid mesothelioma referred to as sarcomatous, spindle or diffuse malignant fibrous mesothelioma.
About 20% of pleural tumors are sarcomatoid, while only 1% of peritoneal mesothelioma are sarcomatous.
Biphasic Cell Subtype
Biphasic mesothelioma refers to tumors that contain epithelial and sarcomatoid cells. Life expectancy after diagnosis with biphasic mesothelioma depends upon which cancer cell appears most frequently in the patient.
More epithelioid cells generally mean a better prognosis. If the tumor is primarily sarcomatous, it is harder to treat, and life expectancy is shorter.
Around 30% of pleural and 25% of peritoneal tumors are biphasic.
Sarcomatoid, mixed (biphasic) and epithelial express themselves within the patient in a different manner. It’s important to understand which of these three types the physician is dealing with in a patient.Dr. Sugarbaker
Rare Mesothelioma Cell Subtypes
Rare cell types of mesothelioma are typically further subtypes of the standard cell types: sarcomatoid, epithelioid and biphasic. These are more irregular forms of mesothelioma that doctors may find difficult to diagnose. These variations have slightly different characteristics that affect the mesothelioma patient’s prognosis and life expectancy.
For example, patients with sarcomatoid tumors tend to have lower life expectancies than patients with epithelial tumors. However, some patients with the lymphohistiocytoid subtype of sarcomatoid cells have survived as long as six years.
According to a 2020 study published in Translational Lung Cancer Research, researchers continue to debate whether to reclassify lymphohistiocytoid as a form of epithelioid mesothelioma.
- Well-differentiated papillary cells commonly occur in peritoneal mesothelioma. Only a handful of cases exist of people with other mesothelioma cancer locations.
- Small cell mesothelioma is another cell type that occurs more commonly in the abdomen.
- A little more than half of the cases of deciduoid mesothelioma occur in the abdomen. Slightly less than half arise in the lung lining.
- Cystic and papillary cells are more common in peritoneal mesothelioma.
- Desmoplastic and lymphohistiocytoid are more common in pleural mesothelioma.
- Adenomatoid cells can occur in both malignant mesothelioma and benign mesothelioma.
- Heterologous cells are present in tumors that may also contain bone, cartilage and soft tissue.
For patients with rare cellular subtypes, treatment is similar to the overarching cell type. For example, patients with lymphohistiocytoid tumor cells are likely to respond well to immunotherapy, a treatment effective against sarcomatoid cells.
Treatment by Type of Mesothelioma
Your specific mesothelioma diagnosis will influence your treatment options. Different treatments may be available depending on the location of your cancer and possibly the cell type.
To find the proper treatment for each patient, doctors consider many factors, such as cancer stage, cell type, patient’s age and overall health. The patient’s provider will also evaluate the patient’s desires and needs going into treatment.
Surgery is an appropriate option for early-stage patients who are younger and in good health. Chemotherapy, radiation therapy and immunotherapy are available to a broader range of patients, and a mesothelioma treatment plan is often multimodal, involving multiple therapies.
The recommended surgeries for pleural mesothelioma are extrapleural pneumonectomy or pleurectomy and decortication. Extrapleural pneumonectomy involves the removal of the cancerous lung and associated structures. Pleurectomy removes the entire pleura, and decortication consists of removing visible tumors from the chest cavity.
Surgeons remove peritoneal tumors with a peritonectomy, which involves removing the peritoneal tissue in the abdomen. The surgery for pericardial tumors is a pericardiectomy, which removes the protective sac surrounding the heart. For testicular mesothelioma, surgeons perform an inguinal orchiectomy.
A combination of cisplatin and Alimta (pemetrexed) is the most effective regimen against pleural mesothelioma. Combinations of gemcitabine, Alimta, mitomycin and carboplatin are most effective against peritoneal mesothelioma.
No particular chemotherapy regimen is consistently effective for pericardial or testicular mesothelioma. The two latter types of mesothelioma are so uncommon and do not have a standardized treatment recommendation.
A mesothelioma expert oncologist can devise the best treatment plan based on their experience and your overall health in these rare situations.
In pleural mesothelioma, specialists use radiation therapy with and without surgery. Radiation therapy isn’t a typical treatment for peritoneal mesothelioma due to its tendency to damage vital organs throughout the abdomen.
However, some doctors have successfully used radiation therapy to treat pericardial and testicular mesothelioma.
The goal of palliative therapy is to alleviate pain and other symptoms of mesothelioma. Palliative care can significantly improve the quality of life and well-being of mesothelioma patients.
For example, palliative procedures include thoracentesis to drain fluid around the lungs and paracentesis to remove fluid from the abdomen. These procedures can alleviate pain and pressure and make breathing and eating easier for you.
Common Questions About Types of Mesothelioma
- What is the difference between mesothelioma cell types and tumor location?
Tumor location refers to where the cancer first began growing such as the lining of the lungs or abdomen. Cell type refers to the type of cells that make up the tumors.
- How does the type of mesothelioma impact prognosis and treatment?
The type of mesothelioma a patient has impacts which treatments will be recommended and their overall prognosis. Mesothelioma treatment is complex, and many other factors influence treatment options and prognosis such as the stage of the cancer.
- How can I find a mesothelioma specialist for my type of mesothelioma?
You can speak with a Patient Advocate to find a mesothelioma specialist who has experience treating your type of mesothelioma.
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