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Types Of Mesothelioma: List, Meaning, Facts, Causes, Signs And Symptoms, Diagnostic Process, Stages, And Treatment Option

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Types Of Mesothelioma: List, Meaning, Facts, Causes, Signs And Symptoms, Diagnostic Process, Stages, And Treatment Option

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Types Of Mesothelioma

There are four types of mesothelioma defined by tumor location. The most common types of mesothelioma are pleural mesothelioma (lung lining) and peritoneal mesothelioma (abdominal lining). There are also three mesothelioma cell types. Epithelioid is the most common cell type.

The four types of mesothelioma are classified by where tumors develop in the body. The most common types are pleural and peritoneal mesothelioma. Pericardial and testicular mesothelioma account for less than 2% of cases combined.

Tumors form in the mesothelium, which is the thin protective lining that covers the lungs, abdomen, heart and testes. The mesothelium goes by different names depending on the parts of the body it covers. The pleura surrounds the lungs and chest wall, the peritoneum covers the abdominal cavity and organs, the pericardium covers the heart, and the tunica vaginalis lines the testes.

Prognosis, symptoms and treatment options vary by mesothelioma type.

Mesothelioma Types by Tumor Location

Mesothelioma types vary 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 regularly 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.

Below ae the list and explanation of the four types of Mesothelioma

Pleural Mesothelioma

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Meaning Of Pleural Mesothelioma

Pleural mesothelioma is the kind of cancer that forms in the lining of the lungs. It is due to the exposure of asbestos. Symptoms include shortness of breath, chest pain, cough and fluid in the lungs. Malignant pleural mesothelioma makes up 75% of mesothelioma cases.

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Malignant pleural mesothelioma is a type of rare cancer that develops in the pleura, the thin membrane that lines the lungs and chest cavity. When asbestos fibers get lodged in the lining of the lungs they can cause inflammation and scarring. The tumors that form in the pleura are known as pleural mesothelioma.

Pleural mesothelioma accounts for 75% of cases. It forms on the soft tissue covering the lungs. Multimodal therapy combining two or more therapies remains the best treatment approach.

Pleural is the most common mesothelioma type. About 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 came out with the best outcome for early-stage pleural mesothelioma, but doctors often diagnose patients after symptoms show in later stages. Typical symptoms can be shortness of breath, chest pain, weight loss, fever and fatigue.

Most patients are allowed for chemotherapy or radiation therapy. Based on health history, many are also allowed for clinical trials or immunotherapy. These therapies can add months or years to life expectancy. With quick and regular treatment, patients can live for three or more years at stage 1 versus approximately 12 months at stage 4.

Facts About Pleural Mesothelioma

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  • Pleural mesothelioma is the most common type of mesothelioma cancer.
  • Symptoms of pleural mesothelioma include shortness of breath, chest pain, dry cough, wheezing and fatigue.
  • Pleural mesothelioma is not curable. It is managed with cancer treatment, which can improve life expectancy and alleviate symptoms.
  • The average life expectancy with pleural mesothelioma is four to 18 months, and the five-year survival rate is 10%.

Doctors discover approximately 3,000 new patients with pleural mesothelioma every year in the United States.

Causes Of Pleural Mesothelioma

The cause of pleural mesothelioma is exposure to asbestos fibers, which a person can inhale or sniffed into the lungs. After the microscopic mineral fibers are inhaled, their sharp, pointed shape causes them to lodge in the lungs and gradually migrate into the pleural lining.

Over many years, these fibers cause irritation, chronic inflammation and genetic changes in DNA that turn healthy pleural cells cancerous. It usually takes from 20 to 50 years for malignant pleural mesothelioma to develop after a person’s first exposure to asbestos. Because of this latency period, the disease usually affects people older than 75.

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The pleural lining of the lungs consists of two layers:

  • The outer layer lines the entire chest cavity under the ribs.
  • The inner layer covers and protects the surface of the lungs.

A malignant tumor can develop on either layer and may spread to the other layer. As nodules develop on the pleural surface, they grow to form tumor masses around the affected lung. The tumors can also lead pleural fluid to accumulate in the chest cavity.

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The combination of tumor mass on the lung and a collection of pleural fluid increases pressure in the chest and prevents the lung from expanding, which causes breathing difficulties.

When abandoned untreated, malignant cells can increase and damage nearby healthy tissues or spread through the blood and lymph system, forming new tumors on distant organs.

Signs And Symptoms Of Pleural Mesothelioma

People with pleural mesothelioma have symptoms that make it almost impossible to breathe, such as shortness of breath and chest pain, but they also encounter struggling swallowing and a dry, raspy cough. For many people, mesothelioma symptoms are not noticeable until the cancer is in a later stage.

Pleural mesothelioma symptoms can include:

  • Shortness of breath
  • Chest pain or painful breathing
  • Persistent dry or raspy cough
  • Coughing up blood
  • Difficulty swallowing
  • Pain in the lower back or rib area
  • Unexplained weight loss and fatigue
  • Swelling of the face or arms
  • Night sweats or fever
  • Lumps under the skin on the chest
  • Fluid in the lungs

Patients hardly mention weight loss and fatigue during their early doctor visit. These symptoms might only show if the cancer is advanced. Some patients also develop swelling of the face or arms, back pain or nerve pain.

Several pleural cancer symptoms and conditions may show a person’s story of asbestos exposure and the potential of a mesothelioma diagnosis. This includes pleural plaques, pleural effusions and pleural thickening.

Pleural Plaques

Small areas of thickening on the pleura are the most common sign someone has a history of asbestos exposure. Pleural plaques are not cancerous and usually do not cause symptoms, but they might show an high risk for cancer.

Pleural Effusion

Irritation from asbestos fibers can lead to excess fluid to build up between the two layers of the pleura. This condition, called pleural effusion, is present in many pleural mesothelioma cases. A little fluid between the pleural layers is healthy. Too much puts pressure on the lungs, causing chest pain that worsens when you cough or take deep breaths.

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Pleural Thickening

When large areas of the pleura stiffen because of scarring, it may become difficult and painful to breathe. Pleural thickening around both lungs is often a sign of significant asbestos exposure. Repeated episodes of pleural effusion can cause the pleural thickening to worsen as scar tissue collects.

Asbestosis

Asbestosis is a pulmonary disease marked by progressive scarring of lung tissue caused by asbestos exposure. This condition does not affect the pleural lining. There is an advanced risk for someone diagnosed with asbestosis to develop asbestos-related lung cancer rather than pleural mesothelioma, but the latter has been documented in medical literature.

Diagnostic Process For Pleural Mesothelioma

The diagnostic process for pleural mesothelioma starts when a doctor evaluates the initial symptoms of the disease. Chest pain and breathing difficulty warrant a chest X-ray, revealing fluid buildup or tumors around a lung. A primary care physician refers a patient to a specialist when they require further testing.

Specialists must use advanced imaging scans and tissue biopsies to confirm a pleural mesothelioma diagnosis. In addition to identifying cancer, determining the cancer stage and cell type is vital to creating an effective treatment plan.

Cell Types

Tissue biopsies recognize the three various cell types that can make up pleural mesothelioma tumors, and this information is important in deciding treatment plans. Most cases of malignant mesothelioma are diagnosed with a high number of epithelial cells, also called epithelioid cells. This cell type responds best to cancer treatment.

The least common cell type is sarcomatoid mesothelioma. It is aggressive and challenging to treat, and most of these cases occur in pleural patients.

The biphasic cell type is a blend of these two cell types. How it responds to treatment depends on the ratio of cells. If more epithelioid cells are present, it will respond better than tumors composed of more sarcomatoid cells.

Several pleural cancer symptoms and conditions may specify a person’s history of asbestos exposure and the potential of a mesothelioma diagnosis, including pleural plaques, pleural effusions and pleural thickening.

In a 2020 study published in Case Reports, researchers discovered a case of pleural mesothelioma through testing of metastatic gastric and colonic polyps identified in imaging scans. These polyps were the only sign that the patient had pleural mesothelioma.

Stages Of Pleural Mesothelioma

Pleural Mesothelioma Stages

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Pleural mesothelioma staging is how doctors get to know the development of cancer in a patient’s body. The stage determines what treatment options doctors might choose to prescribe.

The International Mesothelioma Interest Group created the TNM staging system, the most widely used staging system for pleural mesothelioma.

Stages 1 and 2, which are regarded as the early stages, show localized tumors. Stages 3 and 4, which denote the later stages, classify spreading tumors.

When doctors diagnose patients in stages 1 or 2, they have more options for treating pleural mesothelioma, involving surgery, which offers the best chance at increased survival. In the later stages, treatment options are limited since cancer has begun to spread throughout the body.

Mesothelioma Life Expectancy by Stage for Patients Eligible for Surgery

STAGE TIME PROGRESSION
Stage 1 22.2 months Tumors remain localized in and around the tissue lining of one lung
Stage 2 20.0 months Cancer cells are entering nearby lymph nodes
Stage 3 17.9 months Cancer has spread to nearby tissues and distant lymph nodes
Stage 4 14.9 months Cancer has spread to distant organs

Pleural Mesothelioma Treatment Options

Quality pleural mesothelioma treatment options can include surgery, radiation therapy and chemotherapy.

Chemotherapy is the most major type of pleural treatment. Doctors often prescribe a combination of medicines known as multimodal therapy if they diagnose a patient early in the disease process. These treatments can improve symptoms, such as chest pain and breathing difficulties, and improve survival.

Patients can access these therapies at top cancer centers across the nation that specialize in pleural mesothelioma treatment.

5 Tips When Considering Treatment

  1. Set clear treatment goals. What’s most important to you, longevity or quality of life?
  2. Research each treatment option and ask your doctor as many questions as you can think of.
  3. Determine where you will receive recurring treatments such as immunotherapy infusions. Will you have to travel far and how often?
  4. Consider the side effects of suggested chemotherapy drugs and how it may impact your quality of life.
  5. Seek a second opinion. Surgery may still be an option, or you may be eligible for a clinical trial.

The following are the Pleural Mesothelioma Treatment Options:

Chemotherapy: The most common pleural mesothelioma treatment is chemotherapy. It makes use of one or more drugs, usually a mixture of cisplatin or carboplatin and pemetrexed (Alimta), to eliminate cancer cells or avoid them from reproducing. Recent advances have improved how well patients respond to chemotherapy, but success rates remain low overall.

A new method of delivering chemotherapy known as heated intrathoracic chemotherapy, also called HITHOC, is applied directly to the pleural cavity following surgery. The chemotherapy drugs are heated before being circulated throughout the pleural cavity.

Surgery: Surgery is used to diagnose the cancer, relieve symptoms and eliminate tumors. A common diagnostic surgery includes thoracoscopy, which is a minimally invasive procedure to collect tumor tissue samples for biopsy testing.

Patients diagnosed with later stages of disease may benefit from therapeutic surgeries to control symptoms. A thoracentesis is a minimally invasive surgery that removes fluid from the pleural cavity through a tube inserted through small incision in the chest. A pleurodesis also removes pleural fluid, but it takes the procedure a step further by eliminating the pleural space by fusing the two layers of the pleura together. This prevents the recurrence of fluid buildup known as pleural effusion.

Pleural mesothelioma cancer patients diagnosed in the early stages benefit the most from tumor-removing surgery. The goal of tumor-removing surgery is to eliminate cancer and avoid it from recurring as long as possible.

The two most common tumor-removing surgeries for pleural mesothelioma are extrapleural pneumonectomy and pleurectomy and decortication. A pleural mesothelioma cancer specialist can tell if you are eligible for surgery and advise you on which procedure might be the best option for your diagnosis.

Extrapleural Pneumonectomy: A more aggressive surgical option, the extrapleural pneumonectomy removes the pleura, the entire cancerous lung, the diaphragm and the heart sac (pericardium).

Pleurectomy and Decortication: A pleurectomy and decortication, also known as a P/D or radical pleurectomy, involves removing the tumors and affected pleura (lining of the lung).

Radiation Therapy

Targeted radiation can destroy cancer cells and reduce tumor size. Radiation therapy cannot heal pleural mesothelioma completely, but it is an effective way to manage chest pain. Radiation can also assist in preventing cancer recurrence after surgery.

External beam radiation therapy is the most common form of radiation therapy for pleural mesothelioma. Sessions are fast, painless and only take a few minutes.

Emerging Treatments

Emerging treatments include novel therapies such as immunotherapy, gene therapy and photodynamic therapy to treat pleural mesothelioma. Researchers are refining these treatments to fight cancer more effectively butcausing fewer side effects for the patient.

You might be able to receive experimental therapy through a pleural mesothelioma clinical trial. Some patients may be eligible for immunotherapy drugs and other emerging treatments through compassionate use programs.

Best Mesothelioma Doctors in the U.S.

Doctors who specialize in malignant pleural mesothelioma include oncologists, surgeons and other experts who have years of experience treating this rare disease.

Many of these physicians offer unique services such as clinical research trials and mesothelioma support resources. Working with a top mesothelioma specialist provides the best chance at improving prognosis and quality of life.

  • Dr. Jacques Fontaine (Thoracic Surgeon): Dr. Jacques Fontaine is the Director of the Mesothelioma Research and Treatment Center at Moffitt Cancer Center in Tampa, Florida. He specializes in minimally invasive thoracic surgery including robotic surgery. Fontaine became a pleural mesothelioma specialist while studying under Dr. Sugarbaker at the International Mesothelioma Program at Brigham & Women’s Hospital.
  • Robert B. Cameron (Thoracic Surgeon): Dr. Robert B. Cameron developed a lung-sparing surgery for pleural mesothelioma that not only extends survival but offers greater quality of life by preserving the lung. Cameron’s surgery has a lower risk of complications and studies report longer survival times.
  • Abraham Lebenthal (Thoracic Surgeon): Dr. Abraham Lebenthal is a respected thoracic surgeon who treats pleural mesothelioma patients at the Dana-Farber Cancer Institute. Lebenthal also teaches at Harvard Medical School.

Medical Prognosis for Pleural Mesothelioma

The average pleural mesothelioma prognosis, or the expected course and outcome for the disease, is poor for most patients because this cancer progresses quickly and is resistant to many existing therapies.

The life expectancy for pleural mesothelioma is often less than 18 months, but it depends on many factors.

Accurately forecasting an individual’s prognosis is challenging because this disease is complex. Each person responds differently to treatment.

Pleural Mesothelioma Survival Rate by Year:
YEAR(S) AFTER DIAGNOSIS SURVIVAL RATE
1 year 73%
3 years 23%
5 years 12%
10 years 4.7%

Factors That Affect Your Prognosis

  • Stage: The most important factor in a mesothelioma prognosis is the stage of the disease at diagnosis. An early-stage cancer offers a better chance of long-term survival than a late-stage cancer.
  • Cell Type: The cell type of mesothelioma tumors also greatly influences prognosis.
  • Age: Younger patients tend to live longer.
  • Gender: Women tend to live longer with the disease than men.
  • Pleural Fluid: Higher amounts of pleural fluid in the chest are associated with a poorer prognosis.
  • Patient Activity Level: More active patients have a better prognosis.
  • Cancer Recurrence: A recurrence of mesothelioma is associated with a poorer prognosis.

Improving Your Prognosis

By taking proactive steps, it is possible to live longer and better with malignant pleural mesothelioma. Some patients even achieve remission after treatment and live for years with almost no cancer symptoms.

The inspiring stories of mesothelioma survivors reveal many ways to confront the challenge of cancer. However, many survivors make the same basic choices to promote their well-being.

Tips for Living Longer with Pleural Mesothelioma

Following the advice of your physician and taking steps toward a healthy lifestyle, such as exercise and a mesothelioma diet, can assist to improve your prognosis and extend life expectancy.

  • Seek treatment from an experienced mesothelioma specialist
  • Improve your lifestyle to boost your overall health
  • Access the latest therapies through a clinical trial
  • Use palliative and complementary medicine to improve your quality of life

Expectation for a Mesothelioma Total Treatment

There is no definitive total treatment for pleural mesothelioma. However, a combination of surgery, chemotherapy and radiation therapy have allowed patients to improve their prognosis. Even patients who are not allowed for surgery have survived for years after their diagnosis, thanks to experimental clinical trials and emerging treatments such as immunotherapy.

Researchers around the world have dedicated their careers to finding more effective treatments with the goal of curing pleural mesothelioma and diagnosing the cancer at an earlier stage.

  • Emily Ward was diagnosed in 2012 after working for 43 years as a registered nurse. She put her medical background to work and found the top mesothelioma specialists available to treat her cancer, including the late Dr. David Sugarbaker. She underwent a pleurectomy and decortication, three rounds of chemotherapy and immunotherapy with Keytruda, Opdivo and Yervoy. Ward is enjoying a good quality of life doing many of the things she did before her diagnosis.
  • Tim Crisler passed the 17-year mark as a pleural mesothelioma survivor in 2019. He credits his lung-removing extrapleural pneumonectomy surgery for his long-term survival. He still deals with residual pain, but he has been able to travel the country on his beloved Harley-Davidson since his diagnosis in 2002. “I have absolutely no issues with mesothelioma cancer today,” he said. “That hasn’t touched me in a long time.”
  • Sallie Morton survived pleural mesothelioma for four years while foregoing conventional treatment. She wasn’t eligible for surgery when she was diagnosed in 2013 at the age of 87, and she declined chemotherapy after seeing too many friends deal with the harsh side effects of the drugs. She did it her way, surprising doubters and hosting events and parties to the end. “She’s one gutsy broad,” is how she often described herself.

Common Questions About Pleural Mesothelioma

Question: How does asbestos affect the lungs?

Answer: Damage to asbestos-containing materials releases asbestos fibers as dust. When inhaled, these fibers become trapped in the pleural lining of the lungs. The asbestos fibers trigger an inflammatory response by the immune system. Over the course of decades, this process leads to tissue scarring and DNA damage that causes mesothelioma.

Question: What are the symptoms of pleural mesothelioma?

Answer: The symptoms of pleural mesothelioma include shortness of breath, chest pain, dry cough, wheezing, respiratory complications, fever and night sweats. Other symptoms may develop as the disease progresses.

Question: Is pleural mesothelioma curable?

Answer: Pleural mesothelioma is incurable at all four stages. Clinical trials are providing hope for a cure as they test innovative treatments including immunotherapy and gene therapy.

Question: How long do pleural mesothelioma patients live?

Answer: Most pleural mesothelioma patients have a life expectancy of one year. Patients who are diagnosed early and qualify for aggressive treatment live an average of 22.2 months, and some live for many years.

Peritoneal Mesothelioma

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Meaning Of Peritoneal Mesothelioma

Peritoneal mesothelioma is a type of malignant cancer that is caused by ingested asbestos fibers. It develops in the peritoneum, the thin layer of tissue lining the abdomen. Symptoms of peritoneal mesothelioma include abdominal pain, nausea and swelling in the abdomen (ascites).

Peritoneal mesothelioma accounts for less than 20% of cases. It spreads on the lining surrounding the abdomen. This type responds best to a combination of surgery and heated chemotherapy.

Peritoneal mesothelioma is a rare asbestos-related cancer that forms on the abdominal lining, known as the peritoneum. An even rarer form of this cancer spread in a layer of the abdominal membrane, the omentum, that includes the stomach and other organs.

Peritoneal mesothelioma is also known as abdominal mesothelioma or mesothelioma of the stomach.

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.

Facts About Peritoneal Mesothelioma

  • Early symptoms include abdominal swelling and fluid buildup (known as ascites).
  • Peritoneal mesothelioma accounts for less than 20% of all mesothelioma cases.
  • Surgery with heated chemotherapy is the most effective treatment, helping some patients survive for more than five years.

The initial cause of peritoneal mesothelioma is swallowing airborne asbestos fibers or drinking contaminated water. The digested fibers become trapped in the peritoneum.

Causes Of Peritoneal Mesothelioma

The major cause of malignant peritoneal mesothelioma is ingesting asbestos fibers. Current research suggests that asbestos fibers travel from the digestive tract and remain trapped in the abdomen, causing cancer after years of inflammation.

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Process of Peritoneal Mesothelioma Development

Like other asbestos-related diseases, peritoneal mesothelioma develops over a long period of time. The trapped asbestos fibers cause inflammation and irritation, damaging and degrading DNA.

Damaged DNA can lead to abnormal and unregulated cell growth. These errors cause tumors to form, and the tumors can grow uncontrollably and spread to other areas of the body.

HOW PERITONEAL MESOTHELIOMA DEVELOPS

  • Swallowed asbestos fibers pass through the stomach and digestive system.
  • Ingested asbestos fibers reach the lymphatic system and travel to the peritoneum.
  • Fibers become stuck in the peritoneum, irritating cells and damaging DNA for years.
  • The irritated cells become inflamed, form scar tissue and thicken the peritoneal lining.
  • Years of inflammation and scar tissue formation cause excess abdominal fluid buildup.
  • After prolonged damage, malignant tumors begin to form on the diseased peritoneum.
  • As tumors grow, they put pressure on vital organs and can spread to form new tumors.

Research on peritoneal mesothelioma is still ongoing. Other sources of this disease may include abdominal radiation and fibrous minerals besides asbestos, such as erionite.

Signs And Symptoms of Peritoneal Mesothelioma Cancer

Early symptoms of peritoneal mesothelioma include bloating, pain or tenderness around the abdomen, and digestion issues such as constipation or diarrhea.

Peritoneal Mesothelioma Symptoms Include:

  • Abdominal pain
  • Abdominal swelling (ascites)
  • Night sweats
  • Nausea
  • Fever
  • Unexplained weight loss
  • Fatigue
  • Diarrhea
  • Constipation
  • Loss of appetite

These symptoms tend to imitate many other abdominal disorders. An accurate diagnosis often needs a mesothelioma specialist and a biopsy sample.

Anyone with a history of asbestos exposure should observe their health and see their doctor for new or worsening symptoms.

Diagnostic Process For Peritoneal Mesothelioma

Imaging scans, blood tests and biopsies are used to make an abdominal mesothelioma diagnosis.

  • Imaging scans show the size and location of tumors.
  • Blood tests reveal certain biomarkers associated with cancer.
  • Biopsies show what kind of cancerous cells are present.

Patients will also go through a thorough examination of their medical history, occupational history and overall physical condition.

While every test serves a contributing purpose to the diagnostic process, the only way to confirm a peritoneal mesothelioma diagnosis is with a biopsy.

Biopsies are samples of tumor tissue that doctors examine under a microscope in a lab. Medical professionals, called pathologists, perform tests on the biopsy sample to reveal the kinds of cancerous cells within the tumors. They then summarize the results in a pathology report.

Diagnosing Cell Types of Peritoneal Mesothelioma

The pathology report consists of information about which cellular subtype of peritoneal mesothelioma one have.

Common Cell Types

  • Epithelioid cells: are the most common, making up 75% of cases.
  • Biphasic Cells: Second-most common type, making up 25% of cases. A mix of epithelioid and sarcomatoid cells.

Epithelioid peritoneal mesothelioma can grow in four different patterns: Papillary, tubular, diffuse and deciduous. Papillary is the most common and often appears alongside the others.

Pure sarcomatoid tumors are rare in peritoneal mesothelioma. Specialists have only diagnosed about 30 cases of this variant since 2006. Sarcomatoid cells usually occur alongside epithelioid cells to form the biphasic subtype.

Rare Cell Types

  • Well-Differentiated Papillary Mesothelioma: This variant is uncommon with less potential for malignancy and typically presents in younger women.
  • Multicystic Mesothelioma: This subtype often recurs after surgery and does not have a definitive link with asbestos exposure.
  • Desmoplastic Mesothelioma: This tumor type can present as nodules or a mass-like peritoneal thickening, ascites or lymph node disease.
  • Lymphohistiocytoid Mesothelioma: A dense lymphatic infiltrate characterizes this variant.
  • Pure Sarcomatoid Mesothelioma: The rarest and most lethal form of peritoneal mesothelioma, this subtype is associated with aggressive metastasis.

Additionally, omental mesothelioma is a rare variant in the omentum, a part of the peritoneum surrounding the stomach and other abdominal organs. The omentum is a layer of fatty tissue that plays a role in immune function and metabolism.

Misdiagnosis

Because this cancer is rare, doctors lacking experience with the disease often misdiagnose abdominal mesothelioma patients with more common illnesses that share similar symptoms. Peritoneal mesothelioma misdiagnosis can delay proper treatment.

If you have a history of asbestos exposure, the best way to ensure an accurate diagnosis is to schedule an appointment with a mesothelioma specialist. Doctors who specialize in peritoneal mesothelioma have the knowledge and tools needed to make a precise diagnosis.

Stages of Peritoneal Mesothelioma

There is no official staging system for peritoneal mesothelioma. Instead, peritoneal mesothelioma experts spent decades adapting other staging systems for their use.

Historically, the TNM staging system used in other cancers has not been a good fit for peritoneal mesothelioma because of its diffuse growth pattern. However, a research report in 2020 suggested that oncologists adapt the TNM system for peritoneal mesothelioma into three stages.

3 Stages of Peritoneal Mesothelioma

  • Stage 1: Cancerous tissue is minimal, tumors are only within the abdominal lining and lymph nodes are free of cancer.
  • Stage 2: Cancerous tissue is moderate and tumors have not spread outside the lining or into lymph nodes.
  • Stage 3: Cancerous tissue is more extensive and tumors may have spread outside the peritoneal lining or into lymph nodes.

There is no precise fourth stage in the adapted TNM system for peritoneal mesothelioma. Many doctors consider stage 4 to involve extensive tumors that have spread to distant sites throughout the body.

Peritoneal Cancer Index

The Peritoneal Cancer Index is a diagnostic tool that doctors use to assess tumor location and spread in the abdomen. It assist them to know the approximate mesothelioma stage and appropriate treatments.

This index divides the abdomen into 13 parts. Doctors allocate a number (one to three) to each area based upon the most sizable tumor in that area. The Peritoneal Cancer Index score is the sum of the individual scores from the 13 regions. A patient’s maximum score is 39 (13 times three).

Lower index scores mean the patient may qualify for surgery. Index scores above 20 indicate cancer has spread too far and the patient likely won’t respond well to surgery.

Peritoneal Mesothelioma Treatment Options

The most effective peritoneal mesothelioma treatment option is surgery with heated chemotherapy, known as hyperthermic intraperitoneal chemotherapy, or HIPEC. However, chemotherapy alone is the treatment most used for peritoneal mesothelioma.

More than 60% of patients are ineligible for surgery with heated chemotherapy because of the procedure’s intensity. Cytoreductive surgery can take several hours and may involve removing large portions of the intestines or whole organs.

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Chemotherapy: Patients who are not eligible for surgery with heated chemotherapy can receive systemic chemotherapy alone. Chemotherapy drugs can shrink peritoneal mesothelioma tumors and reduce the growth and spread of cancer. Chemotherapy drugs that are effective against peritoneal mesothelioma include pemetrexed, cisplatin, carboplatin and gemcitabine.

U.S. Navy pilot and peritoneal mesothelioma survivor Jim Madaris decided to seek out the HIPEC procedure after his diagnosis in 2013. The surgery took 14 hours and removed his gallbladder, spleen and parts of his intestine. Madaris extended his life by four years, allowing him to find joy in the little things and see his youngest child graduate high school.

Cytoreductive Surgery with Heated Chemotherapy

Cytoreductive surgery, also known as a peritonectomy, attempts to remove as much cancer as possible. Then, heated chemotherapy is applied locally to the abdomen before the surgery ends. Doctors only perform this method on a case-by-case basis. A 2019 study reported that more than 65% of the patients who underwent this procedure lived five years or longer.

Palliative Treatment Options

In addition to anti-cancer treatments, many peritoneal patients receive palliative care to control symptoms and improve quality of life. For example, a paracentesis procedure may be suggested to certain peritoneal patients to drain excess fluid from the abdomen.

A palliative care specialist prescribes medications to control pain and side effects. They may refer patients to physical or occupational therapy or recommend complementary therapies.

Best Peritoneal Doctors

  • Dr. Paul H. Sugarbaker (Surgical Oncologist): Dr. Paul Sugarbaker is the country’s leading expert on peritoneal mesothelioma. He developed the widely renowned cytoreductive surgery and heated chemotherapy technique that changed the landscape of peritoneal cancer treatment. Many people with peritoneal mesothelioma are alive today because of Sugarbaker’s innovations.
  • W. Charles Conway (Surgical Oncologist): Dr. W. Charles Conway is an expert in peritoneal mesothelioma and the Director of Surgical Oncology at Ridley-Tree Cancer Center in Santa Barbara, California. He specializes in minimally invasive robotic surgery and heated chemotherapy for peritoneal mesothelioma.
  • J.F. Pingpank Jr. (Surgical Oncologist): Dr. J.F. Pingpank Jr. is a peritoneal mesothelioma expert who advocates for regional therapy. The approach applies treatment locally around the cancer to limit damage to the rest of the body. Pingpank specializes in cytoreductive surgery and heated chemotherapy.

Medical Prognosis And Life Expectancy Of Peritoneal Mesothelioma

The prognosis for peritoneal mesothelioma is generally poor for patients who are not allowed for surgery. Peritoneal mesothelioma life expectancy is approximately 12 months for patients who undergo chemotherapy alone. The median survival of untreated peritoneal mesothelioma patients is six months.

Although, the prognosis for surgical candidates is significantly better. Surgery and heated chemotherapy can improve peritoneal survival by several years.

About half of peritoneal patients who undergo a mixture of cytoreductive surgery with heated chemotherapy have a life expectancy of more than five years.

According to a 2020 study published in the American Journal of Surgical Pathology, women with peritoneal mesothelioma are liable to live longer than men. In the study, 77.8% of women treated with cytoreductive surgery and heated chemotherapy at MD Anderson Cancer Center in Houston survived longer than five years.

Alexis was diagnosed with peritoneal mesothelioma in 2007, had major surgery that removed part of her diaphragm, yet kept her wedding plans in place. She still rides to work on her scooter and refuses to let mesothelioma get in the way of the life she and her husband built. They don’t know how many wedding anniversaries they will have, but they celebrate their time together. “It’s kind of cool now to hear people say, ‘You don’t look or act sick,’” Alexis said.

When averaging short- and long-term survival, women live approximately 13 months on average, and men live roughly six months.

Prognostic Factors

Patients with tumors containing epithelial cells have a longer life expectancy than patients with sarcomatoid or biphasic cells. Epithelial cells tend to respond better to treatments such as chemotherapy.

  • Stage at diagnosis
  • Cell type
  • Tumor grade (how fast it grows)
  • Gender
  • Genetic mutations
  • Treatments selected

The tumor’s grade also impacts prognosis. Tumor grade indicates how quickly tumors are likely to grow and spread, based upon how abnormal the cells appear.

Chemotherapy Survival Rates

Systemic (whole-body) chemotherapy combined with cisplatin and pemetrexed has the following benefits:

  • A response rate of about 30%
  • Average progression-free survival around 11.5 months
  • Median survival around 13 months

Chemotherapy done directly to the peritoneum without surgery has a higher response rate of 47%. When combined with heated chemotherapy given during surgery, the response rate is 84.6%.

Expectation For Peritoneal Mesothelioma Total Treatment

There is no total treatment for peritoneal mesothelioma or any other type of mesothelioma. However, peritoneal patients eligible for surgery and HIPEC typically live longer than five years.

Some patients may be allowed for a repeat surgery with heated chemotherapy if a cancerous mesothelioma recurrence occurs.

In 2017, Dr. Paul Sugarbaker reported increase in survival among patients who received early post-operative chemotherapy and long-term chemotherapy after cytoreductive surgery with heated chemotherapy.

Of the 29 patients who had surgery with HIPEC, post-operative chemotherapy and long-term chemotherapy, 75% lived longer than five years. No patients in the study received intravenous or systemic chemotherapy.

Feelings of depression or loneliness are common among peritoneal mesothelioma patients. Support services such as counselors and support groups are available. Talking to a counselor or joining a support group can help improve quality of life.

The Mesothelioma Center, for example, hosts an online support group that connects mesothelioma patients and families with one another and offers professional guidance from facilitators. Dana Nolan, MS, LMHC, is a licensed counselor and serves as our moderator. Patient advocate and registered nurse Karen Selby is also available to answer medical questions.

Frequently Asked Questions (F.A.Q) About Peritoneal Mesothelioma

Question: What are the symptoms of peritoneal mesothelioma?

Answer: The symptoms of peritoneal mesothelioma include abdominal pain, abdominal swelling or distension, diarrhea or constipation, weight loss and fatigue. Other symptoms may develop as the disease progresses such as fever and night sweats.

Question: Is peritoneal mesothelioma curable?

Answer: Peritoneal mesothelioma is incurable at all four stages. Clinical trials are searching for a cure by testing new treatments including immunotherapy and multimodal therapy.

Question: How long do peritoneal mesothelioma patients live?

Answer: Peritoneal mesothelioma patients have a life expectancy of about one year. However, survival is significantly better for patients who qualify for surgery with heated chemotherapy. Half of the patients who qualify for this procedure live at least five years.

 

Pericardial Mesothelioma

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Meaning Of Pericardial Mesothelioma

Pericardial mesothelioma is a very rare cancer that affects the lining of the heart, known as the pericardium. Only 150 cases of pericardial mesothelioma have been diagnosed. Symptoms of pericardial mesothelioma include difficulty breathing, chest pain and irregular heartbeat.

The pericardial mesothelioma tumor type is exceptionally scarce. 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.

Pericardial mesothelioma accounts for 1% of cases. It forms on the soft tissue around the heart. A multimodal approach of surgery and chemotherapy offers the best chance of survival.

Pericardial mesothelioma is a rare type of cancer that affects the lining of the heart.

Almost all mesotheliomas can be traced to asbestos exposure, but medical researchers continue to study the link between asbestos and cancer of the pericardium. About 25 percent of people with this disease have a history of asbestos exposure. Studies are clear on how inhaled microscopic fibers reach the lungs but less clear on how the fibers may reach the heart.

This form of cancer is most often diagnosed in people between the ages of 50 and 70, with the median age being 55. According to a 2017 study published in the Journal of Clinical Oncology, approximately 60 percent of cases occur in men.

Like the other types of mesothelioma, the disease is typically discovered at a later stage. Symptoms include chest pain, fatigue and shortness of breath. Diagnosis is difficult because symptoms mimic those of other heart disorders.

Pericardial treatment options include surgery, chemotherapy, palliative treatments to control symptoms and rarely radiation therapy. Surgery is the most common treatment with about 46 percent of patients electing surgery. About 37 percent receive chemotherapy and only 8 percent receive radiation therapy because it is risky to apply near the heart. Nearly 25 percent of patients elect no treatment.

A 2021 research study reported that patients with primary pericardial mesothelioma have a median survival of about two months. However, some people have lived for years with this cancer after undergoing surgery or chemotherapy.

To date, less than 150 cases of pericardial mesothelioma are described in medical literature, that’s 1 percent of all known diagnosed mesotheliomas.

Causes Of Pericardial Mesothelioma

The primary cause of pericardial mesothelioma remains a mystery. The causal relationship between asbestos exposure and this type of mesothelioma is not fully understood. Researchers confirm the pleural and peritoneal types are primarily caused by exposure to asbestos, yet the causes of the pericardial type are less definitive.

A 2017 review of medical literature on pericardial mesothelioma reported asbestos exposure in 25 percent of cases. Another 2017 study published in the Annals of Epidemiology reported the majority of patients with pericardial mesothelioma have no history of asbestos exposure.

Signs And Symptoms of Pericardial Mesothelioma

Most pericardial mesothelioma patients experience no symptoms when the cancer first develops, a fact that contributes to a late-stage diagnosis. The symptoms also resemble those of other heart conditions, making the cancer difficult to accurately diagnose. Most symptoms are caused by fluid buildup around the heart and thickening of the pericardial layers.

Most symptoms are caused by the buildup of fluid and the thickening of pericardial layers. The presence of any of these symptoms should be followed by a visit to the doctor with recommended screenings such as an X-ray, CT scan or echocardiogram (ultrasound of the heart).

The following Symptoms may indicate Pericardial Mesothelioma:

  • Difficulty breathing, even when at rest (dyspnea)
  • Heart palpitations or irregular heartbeat (arrhythmia)]
  • Murmurs
  • Cough
  • Shortness of breath when lying flat
  • Chest pain
  • Fatigue
  • Fever
  • Night sweats

If you are going through any of these symptoms, especially heart-related symptoms, schedule an appointment with your doctor immediately. Screening tests like X-rays, CT scans and echocardiograms can usually show the underlying cause of these health problems.

Diagnostic Process For Pericardial Mesothelioma

To diagnose pericardial mesothelioma, doctors make an overall assessment of your symptoms, medical history and current medical condition. Next, you will receive a physical examination, imaging tests and biopsy to determine the location of tumors and confirm whether or not they are cancerous.

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Image Credit: Asbestos.com

When you see a doctor to discuss heart-related symptoms, one of the first diagnostic tests you will receive is an echocardiogram, which is essentially an ultrasound for your heart. This noninvasive test uses sound waves to help doctors see the size and shape of your heart and determine how well it is working overall.

Although an echocardiogram can reveal fluid buildup around the heart and help guide a pericardiocentesis, which is the procedure doctors perform to drain the fluid, other imaging scans are needed to determine if potential tumors are present. If doctors spot abnormal growths, they need to take a fluid or tissue sample and perform a biopsy, which can confirm a mesothelioma diagnosis.

These tumors generally are not localized, and they tend to cover most of the heart. Furthermore, this cancer type accounts for approximately half of all pericardial tumors.

Physical Examination

Unfortunately most cases of pericardial mesothelioma are only found at autopsy. Nonetheless, about 10-20 percent of these cases are diagnosed before a patient dies. Some of the challenges with early and accurate diagnosis of the pericardial type include the small number of people with the disease and the late presentation of symptoms.

Imaging Tests

Computed tomography (CT) scans are effective for detecting pericardial tumors, making them a preferred diagnostic tool. Magnetic resonance imaging (MRI) can also yield successful results in identifying the cancer.

Radionuclide imaging is a detection method involving the injection of gallium, a radioactive material, to identify cancerous cells. It was reportedly used to detect at least one case of this type of cancer. Patterns of radioactivity show possible tumor sites in the human body.

Biopsy

A biopsy is a crucial diagnostic procedure that can be more suitable in determining the cancer’s point of origin. A surgeon will remove tissue or fluid from the pericardium, examine its cells under a microscope to positively confirm the presence of pericardial mesothelioma. According to a 2016 study in the Journal of Cardiac Failure, fluid biopsies often turn up cancer free even when pericardial tumors are present. A tissue sample of the tumor itself is usually required to confirm pericardial mesothelioma.

Misdiagnosis

Because pericardial mesothelioma symptoms such as chest pain, cough and difficulty breathing are shared with various other diseases, it’s crucial to meet with a specialist to confirm your diagnosis. To ensure an accurate diagnosis and prompt treatment, you need to speak with an expert who knows the intricacies of this rare cancer.

Pericardial Mesothelioma is often mistaken for more common heart illness including:

  • Heart failure
  • Coronary heart disease
  • Tuberculosis pericarditis
  • Constrictive pericarditis
  • Cardiac tamponade
  • Intra-atrial myxoma
  • Cardiomyopathy

If you have been diagnosed with any of these conditions, always seek a specialist to confirm the diagnosis. Working with a specialist may get you access to a wider range of treatment options, but it might also lead to improved survival.

Pericardial Mesothelioma Treatments Options

Mesothelioma treatment options are limited because the heart lining rests so closely to the heart itself that most therapies can easily damage this delicate organ. Slightly more than half of patients with pericardial mesothelioma are not ideal candidates for surgery, which is often the most effective treatment for this cancer. However, there are rare cases where the cancer is diagnosed early and surgery can be carried out to remove small, localized tumors.

The primary treatment options for pericardial mesothelioma are pericardiectomy or tumor removal, chemotherapy and palliative treatment such as fine needle aspiration. Radiation therapy is considered minimally effective for this rare cancer and is risky to administer without harming the heart.

Pericardiectomy or Tumor Removal

Patients whose doctors determine they are good candidates for surgery are treated with a pericardiectomy or tumor removal.

A pericardiectomy is the surgical process of removing part or all of the pericardium. A pericardiectomy can relieve pressure and minimize fluid buildup, allowing the heart to continue functioning properly.

Tumor removal, also called tumor resection, can involve removal of the cancer without removing the pericardium. A 2017 review reported longer survival in patients who underwent tumor removal than patients who had a pericardiectomy.

Chemotherapy: The benefits of chemotherapy are minimal for most patients with pericardial mesothelioma. The chemotherapy drugs pemetrexed and cisplatin have shown a survival benefit, and gemcitabine has produced mixed results. A handful of cases have responded well to chemotherapy. One woman lived longer than two years thanks to a combination of the chemotherapy drugs cisplatin, gemcitabine and vinorelbine. Pembrolizumab, a new checkpoint inhibitor form of immunotherapy, has shown some benefit in cases of pleural mesothelioma. It may have some utility in this disease as well.

Palliative Treatment: Palliative treatment options aim to minimize pain and reduce symptoms caused by the buildup of fluid in the pericardium. Essentially, palliative care can improve quality of life and make patients more comfortable.

Palliative treatments can include therapies that relieve pain and pressure around the heart such as a pericardiocentesis or pain medication. A pericardiocentesis removes excess fluid from the pericardium to relieve pressure around the heart.

Medical Prognosis Of Pericardial Mesothelioma

The common prognosis for the pericardial type is poor, compared to that of peritoneal or pleural mesothelioma. Nearly 50 to 60 percent of pericardial patients pass away within six months of receiving a diagnosis. However, this is not the case for everyone.

Hope exists for some patients because researchers have cited positive results after surgery to remove local tumors. Smaller, less impactful benefits have been demonstrated from chemotherapy, except for one case in which a 47-year-old woman was living two years after chemotherapy with cisplatin, gemcitabine and vinorelbine.

A 1995 case report of a 27-year-old woman who underwent surgery and radiation therapy for pericardial mesothelioma states that she had no evidence of recurrence during 28 years of follow-up.

A 1992 case report of a 12-year old boy with pericardial mesothelioma who underwent surgery and chemotherapy says that he remained free of symptoms and showed no sign of disease one year later.

Surgery to remove part of the heart’s lining combined with radiation therapy improved survival in two patients in the late 1960s and early 1970s. One patient lived a year after treatment, and another was alive five years after treatment.

Testicular Mesothelioma

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Meaning Of Testicular Mesothelioma

Testicular mesothelioma is a cancer that forms on the membrane lining the testes. This rare disease, also known as mesothelioma of the tunica vaginalis testis, accounts for less than 5% of all mesothelioma cases.

Testicular mesothelioma accounts for less than 1% of cases. It develops on the tunica vaginalis, which is the lining of the testes. This type responds best to surgery followed by chemotherapy.

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.

Fact Abut Testicular Mesothelioma:
Testicular mesothelioma is a clinically aggressive type of cancer, which means it can spread rapidly. The testicular type tends to recur within a few years, even in cases where tumors are surgically removed.

Causes Of Testicular Mesothelioma

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Unlike pleural or peritoneal mesothelioma, which can easily be traced to asbestos exposure, doctors do not yet understand how asbestos exposure can cause a primary tumor to develop on the tunica vaginalis, the pouch of serous membrane that covers the testes.

It appears as firm, white-yellow nodules on the surface of the tunica vaginalis. The nodules can eventually encase the contents of the scrotum and cause the membrane to thicken.

Local trauma and inflammation also may play a role in its development. Some cases present without any identifiable risk factors.

Medical Prognosis and Survival Rate for Testicular Mesothelioma

Men with this cancer have a significantly better prognosis than most people with other types of mesothelioma.

Nearly 49% of patients survive five years after diagnosis, and 33% survive 10 years, according to a 2019 study published in the journal Urology.

Patients with the biphasic cell type of the cancer were associated with worse overall survival compared to other mesotheliomas and the epithelioid subtypes.

Of the 113 testicular mesothelioma patients in the study, 75% of cases had the epithelioid cell type. Older patients and those with tumors greater than or equal to 4 centimeters in size were associated with a worse survival.

Signs, Symptoms And Diagnostic Process For Testicular Mesothelioma

There is no set of symptoms unique to testicular mesothelioma cancer. Many people with this cancer initially receive the wrong diagnosis because doctors mistake it for a more common condition such as a hernia.

The most common testicular mesothelioma symptom is a hydrocele, a buildup of fluid in the scrotum.

More than half of the patients in the 2019 Urology study had a hydrocele. About one-third of patients had a painless scrotal mass.

Because it is so rare and doesn’t present unique symptoms, testicular mesothelioma is often diagnosed during or after surgery.

The definitive way of confirming a cancer diagnosis is to perform a biopsy. This involves removing a tissue sample from the tumor and sending it to the lab for testing.

Evaluating the sample with a technique called immunohistochemical staining helps doctors determine if the patient has testicular mesothelioma or another disease.

Testicular Mesothelioma Treatment Option

The most common treatment for testicular mesothelioma is surgery. Nearly all of the 113 testicular mesothelioma patients (98.2%) in the 2019 study underwent surgical resection of the tumor.

This method detach the affected testicle and the entire spermatic chord. If the cancer has spread to nearby lymph nodes, doctors will separate them as well with a surgery called lymphadenectomy.

A 2021 research study noted that testicular mesothelioma requires extensive resection including metastatic surgery if complete resection is possible. The study suggested a multimodality treatment plan for the malignancy, similar to a multimodal approach to pleural mesothelioma, offers the best patient outcomes.

Surgery may be followed by chemotherapy with cisplatin and pemetrexed, the longstanding standard-of-care treatment for mesothelioma. Doctors may offer radiation therapy next to kill any remaining cancer cells and prevent the cancer from returning.

Only 5.3% of patients received radiation therapy in the 2019 study. Researchers could not determine if adjuvant therapy has an impact on overall survival, given such a small cohort.

In some cases, testicular mesothelioma is a secondary tumor, with the primary tumor located within the peritoneum (the membrane that lines the abdominal cavity). For situations like this, doctors have to treat the patient for peritoneal mesothelioma as well.

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.

A supplementary form of the disease includes benign mesothelioma, which is neither asbestos-related nor cancerous. Asbestos exposure leads to cancer known as malignant mesothelioma.

Various 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
Epithelioid 50% 75%
Sarcomatoid 20% 4%
Biphasic 30% 24%
Source:Journal of Surgical Research, 2015

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:

epithelial cell

Epithelioid mesothelioma makes up around 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 cells under a microscope.

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 cells under a microscope.

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.

Rare Mesothelioma Cell Subtypes

Rare cell types of mesothelioma are generally further subtypes of the standard cell types: sarcomatoid, epithelioid and biphasic. These are more irregular forms of mesothelioma that doctors may find hard 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 almost the same 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

A particular mesothelioma diagnosis will determine your treatment options. various treatments might be available depending on the location of your cancer and possibly the cell type.

To find the adequate 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.

Surgery:  The approved 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.

Chemotherapy Drugs: 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.

Radiation Therapy: In pleural mesothelioma, specialists use radiation therapy with and without surgery. Radiation therapy isn’t a particular 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.

Palliative Treatment: 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.

Frequently Asked Questions (F.A.Q) About Types of Mesothelioma

Question: What is the difference between mesothelioma cell types and tumor location?

Answer: 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.

Question: How does the type of mesothelioma impact prognosis and treatment?

Answer: 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.

Question: How can I find a mesothelioma specialist for my type of mesothelioma?

Answer: You can speak with a Patient Advocate to find a mesothelioma specialist who has experience treating your type of mesothelioma.

 

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