STAGING PERITONEAL MESOTHELIOMA
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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 contains information about which cellular subtype of peritoneal mesothelioma you have.

Common Cell Types.

Epithelioid cells are the most common, making up 75% of cases.
The biphasic type, which is a combination of epithelioid and sarcomatoid cells, is the second most common. It comprises about 25% of cases.

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 uncommon 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.
Multicystic mesothelioma.
Desmoplastic mesothelioma.
Lymphohistiocytoid mesothelioma.
Pure sarcomatoid mesothelioma.

Additionally, omental mesothelioma is a rare variant that forms in the omentum, a part of the peritoneum surrounding the stomach and other abdominal organs.

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.

Peritoneal Mesothelioma Staging.

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 due to the diffuse pattern of growth. However, a research report in 2020 suggested that oncologists could 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 the location and spread of tumors on the peritoneum. It helps them determine the approximate mesothelioma stage and appropriate treatments.

The index divides the abdomen into 13 parts. Doctors assign 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 that 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, also known as hyperthermic intraperitoneal chemotherapy, or HIPEC. However, chemotherapy alone is the most commonly used treatment for peritoneal mesothelioma.

More than 60% of patients are ineligible for surgery with heated chemotherapy due to 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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Staging Peritoneal Mesothelioma
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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 procedure 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.

Chemotherapy.

Patients who don’t qualify for surgery with heated chemotherapy can receive systemic chemotherapy alone. Chemotherapy drugs can shrink peritoneal mesothelioma tumors and slow 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.

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 recommended 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.

Peritoneal Mesothelioma Life Expectancy and Prognosis

The prognosis for peritoneal mesothelioma is generally poor for patients who don’t qualify for surgery. Peritoneal mesothelioma life expectancy is approximately 12 months for patients who receive chemotherapy alone. The median survival of untreated peritoneal mesothelioma patients is six months.

However, 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 combination 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 tend 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.

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

Prognostic Factors.

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

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.

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 delivered 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%.

Is There a Cure for Peritoneal Mesothelioma?

There is no cure 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 eligible for a repeat surgery with heated chemotherapy if a mesothelioma recurrence happens.

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

Staging Peritoneal Mesothelioma - Peritoneal Mesothelioma Staging
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Final Days of Mesothelioma
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What Happens in the Final Stages of Mesothelioma?

According to the Mayo Clinic, the following symptoms are what happens in the final stages of different types of mesothelioma:

Pleural mesothelioma – The final stages of pleural mesothelioma that surrounds the lungs include painful coughing, shortness of breath, chest pain, unexplained weight loss, and lumps of tissue under the skin in the chest area.
Peritoneal mesothelioma – The final stages of peritoneal mesothelioma that occurs in the abdomen include severe abdominal pain and swelling, nausea, and weight loss.
Pericardial mesothelioma – The final stages of pericardial mesothelioma that occurs around the heart tissue include difficulty breathing and chest pain.
Tunica vaginalis mesothelioma – The final stages of tunica vaginalis mesothelioma that affects testicular tissue may be pain near the tumor or swelling.

How Mesothelioma Develops?

Mesothelioma is a type of cancer caused by exposure to asbestos, which can lay dormant in the body for decades. When mesothelioma cancer begins to develop, it is typically aggressive and deadly. Research from the Annals of Translational Medicine indicates that some people have a greater risk of developing mesothelioma over their lifetime. Mesothelioma is currently linked to asbestos exposure from the mining, use, or processing of asbestos. People who suffered substantial exposure to asbestos include the following:

Workers in asbestos mining plants.
Workers in asbestos processing plants.
Those who live near any asbestos mining or processing plant.
Firefighters and construction workers working in older homes with asbestos.
Workers in industries (prior to the 1980s) that contained asbestos materials including power plants, chemical plants, auto mechanics, shipyards, building construction, boiler workers, insulation workers, military facilities, or home renovation businesses.
Homeowners who self-renovated their houses containing asbestos.
World Trade Center workers following the 9/11 terror attacks.

No Safe Asbestos Exposure.

Research published in the Pakistan Journal of Medical Sciences indicates that workers who suffered exposure to asbestos inhaled asbestos particles that traveled to the lungs or the peritoneal membrane through the lymphatic system. At this point, the asbestos fibers became trapped and eventually led to inflammation, irritation, and cellular changes, resulting in cancer. While researchers are uncertain why asbestos fibers take so long to develop into cancer, the connection between asbestos and mesothelioma cancers appears quite clear.

Life Expectancy.

Mesothelioma is considered a fatal disease. Therefore, the final stages of mesothelioma often result in death. However, some factors can impact the life expectancy of a person diagnosed in the final stages, including whether they are in good overall health, if they were a smoker, their age, how well they responded to treatment for their cancer, and the type of mesothelioma they have. However, statistics from the American Cancer Society show that patients who suffer from the final stages of mesothelioma have just an 8 percent survival rate. This may improve as advancements in medicine and treatments continue.

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INTRAPLEURAL CHEMOTHERAPY MESOTHELIOMA
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Surgery is one of the steps of multimodality approach for the treatment of MPM. Due to anatomical features, microscopically radical (R0) resection is never possible and a Macroscopic Complete Resection (R1) is considered the target for mesothelioma surgeons. Recently, intracavitary therapies have been described with the aim of extending the loco-regional effect of surgery. Different agents might be administered intrapleurally: chemotherapy drugs are the most widely used, but also photodynamic therapy (PDT) showed to lead to satisfactory long-term outcomes; furthermore, immunotherapies and gene therapies have been also reported. Despite promising results, no high-quality evidences are currently available and controlled randomized trials are required to establish the exact role of intracavitary therapies and to standardize the technique.

Keywords: Malignant pleural mesothelioma (MPM), multimodality treatment, surgery, intracavitary therapy

0:08 Abstract
1:10 Introduction
3:08 Intrathoracic therapies
3:36 Chemotherapeutic agents and hyperthermia
7:29 Povidone iodine
8:25 Photodynamic therapy (PDT)
10:45 Intrapleural immunotherapies
12:38 Gene therapy
13:16 Conclusions

INTRAPLEURAL CHEMOTHERAPY MESOTHELIOMA
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Sarcomatoid Mesothelioma
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Sarcomatoid mesothelioma is a rare cell type caused by asbestos exposure. It accounts for approximately 10 – 20% of all mesothelioma diagnoses. Sometimes referred to as spindle cell mesothelioma, sarcomatoid cells are recognized by their oval, spindle shape. This cell type typically forms in separate nodes or lesions and can metastasize, or spread, quickly to distant organs. It is also the most difficult cell type to treat, with an average prognosis of six months.

What Is Sarcomatoid Mesothelioma?

Sarcomatoid mesothelioma is a less common cell type. It accounts for about 10 – 15% of all pleural mesothelioma diagnoses and 4% of peritoneal mesothelioma diagnoses.

There are several defining characteristics that can help identify sarcomatoid cells:

1) An oval, elongated shape with a large nucleus.
2) Multiple nuclei in some cells.
3) Unstructured formation in fibrous nodes or lesions.
4) Distant cell formation that is faster to metastasize than other cell types.

Because sarcomatoid cells are notable for their oblong, spindle shape, sarcomatoid mesothelioma is also referred to as spindle cell or sarcomatous mesothelioma.

Compared to other mesothelioma cell types, malignant sarcomatoid cells are more aggressive. This type of mesothelioma cancer is also considered more difficult to diagnose and treat. Sarcomatoid cells mimic healthy cells and are also frequently misdiagnosed.

Symptoms. - Sarcomatoid Mesothelioma Symptoms.

Malignant sarcomatoid mesothelioma symptoms may take 10 – 50 years to develop. Patients may experience different symptoms depending on where the cancer develops.

Sarcomatoid cells are most common in malignant pleural mesothelioma, which impacts the lining of the lungs. In rare cases, patients may be diagnosed with sarcomatoid peritoneal mesothelioma. This type of mesothelioma develops in the abdominal lining.

Common Symptoms of Sarcomatoid Mesothelioma.

Abdominal or chest pain.
Decreased appetite.
Difficulty breathing (dyspnea).
Excess fluid in the lungs or abdomen.
Fatigue.
Nausea and vomiting.
Persistent cough.
Shortness of breath.
Weight loss.

Because malignant sarcomatoid cells spread quickly, sarcomatoid patients may experience worsening symptoms earlier than patients with epithelioid or biphasic cell types.

Diagnosis - Diagnosing Sarcomatoid Mesothelioma.

Properly identifying mesothelioma cell type is an important aspect of the diagnostic process. Sarcomatoid malignant mesothelioma is difficult to accurately diagnose because of its rarity and resemblance to other conditions.

Diagnosis typically begins with imaging tests, including an X-ray and CT scan. These tests will identify any visible tumors or excess fluid in the chest cavity. If cancer is suspected, doctors may order a blood test. This test identifies biomarkers that can differentiate mesothelioma from other conditions.

A biopsy is the most important step in making an accurate sarcomatoid mesothelioma diagnosis. Cell type can be determined through a tissue biopsy and analyses of the sample, such as immunohistochemistry. This is a staining method that uses specific antibodies to isolate antigens or proteins in the tissue sample.

Through an immunohistochemical stain, a pathologist can identify if a tumor is benign or malignant, as well as determine cell type, stage and prognosis.

Several immunohistochemical markers can indicate mesothelioma. Studies have found stains that test positive for proteins calretinin and D2-40, in combination with the antibody pancytokeratin, are the most accurate indicator of sarcomatoid mesothelioma.

Multiple biopsies may be necessary to determine if a patients’ tumors solely exhibit sarcomatoid cells. In some cases, epithelioid cells may also be present, indicating biphasic mesothelioma. Additional tests may also be needed to make an accurate differential diagnosis.

Common misdiagnoses of sarcomatoid mesothelioma include:

Fibrous pleurisy.
Fibrous tumors.
Fibrosarcoma.
Metastasized renal sarcoma.
Non-Hodgkin’s lymphoma.
Pleural liposarcoma.
Sarcomatoid carcinoma.
Soft tissue sarcomas.

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How To Diagnose Peritoneal Mesothelioma?
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Peritoneal Mesothelioma Treatment.

Peritoneal mesothelioma is often treated with a multimodal approach, which combines conventional treatments like surgery, chemotherapy and radiation therapy.

Traditional chemotherapy and radiation therapy have shown limited success in peritoneal mesothelioma patients, with many clinical trials showing a median survival time of one year. However, some studies indicate success when using chemotherapy during surgery to treat peritoneal mesothelioma.

For patients with advanced stage peritoneal malignant mesothelioma, standard treatments may be used as palliative care. Palliative treatments are used to improve quality of life and extend life expectancy.

Minimally invasive procedures, like paracentesis, are often used palliatively. This procedure removes buildup of fluid and relieves symptoms associated with peritoneal malignant mesothelioma.

Clinical trials are continually testing new treatments for peritoneal mesothelioma. Emerging treatment options include pressurized intraperitoneal aerosol chemotherapy (PIPAC), a chemotherapy applied in a pressurized form. Treating mesothelioma with PIPAC is in early phases and requires further testing. However, the treatment has shown a median life expectancy of approximately 27 months.

Hyperthermic Intraperitoneal Chemotherapy (HIPEC).

For eligible patients, a combination treatment of debulking surgery combined with heated chemotherapy is becoming a standard of care. The heated chemotherapy, known as hyperthermic intraperitoneal chemotherapy (HIPEC), is applied to the abdomen during surgery.

Doctors perform debulking surgery, also known as cytoreductive surgery, prior to HIPEC to remove visible tumors and tissue that have been damaged by mesothelioma cells. This may involve removing the abdominal lining or other organs in order to remove as much of the tumor as possible.

HIPEC is then applied throughout the abdominal cavity to kill any remaining cancer cells. The heated chemotherapy is applied at 104°F – 109°F and consists of a stronger dosage than traditional chemotherapy.

Clinical trials have shown the effectiveness of the treatment, with life expectancy ranging from 53 to 92 months. Data suggests approximately 65% of patients survive five years or longer.

Because HIPEC is a specialized treatment, it is available at select cancer centers. Patients should also be aware of treatment costs as they make treatment decisions. Recent estimates show that surgery with HIPEC can average $40,000.

Common Questions About Peritoneal Mesothelioma.

Is peritoneal mesothelioma curable?
There is no cure for peritoneal mesothelioma. There are treatment options available that may improve life expectancy.

How long do peritoneal mesothelioma patients live?
A peritoneal mesothelioma patient has a life expectancy of two to six years depending on stage at diagnosis.

What are the symptoms of peritoneal mesothelioma?
Some common symptoms of peritoneal mesothelioma are abdominal pain, abdominal swelling, weight loss, fever, fluid in peritoneal cavity, intestinal obstruction, night sweats and hypercoagulability.

Can peritoneal mesothelioma spread to the lungs?
Yes, peritoneal mesothelioma can spread to the lungs. This process is called metastasis.

How is peritoneal mesothelioma diagnosed?
A biopsy is the only way to definitively diagnose malignant peritoneal mesothelioma. Other diagnostic tests like imaging scans and blood tests may be used to confirm the tumors.

What causes peritoneal mesothelioma?
Peritoneal mesothelioma is caused by ingesting asbestos fibers.

How To Diagnose Peritoneal Mesothelioma? - What Is Peritoneal Mesothelioma?
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How To Diagnose Peritoneal Mesothelioma?
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What Is Peritoneal Mesothelioma?

Peritoneal mesothelioma is a form of cancer caused by asbestos. When asbestos fibers are ingested or inhaled, they can become embedded in the lining of the abdomen. Research suggests the fibers reach the abdominal lining through the digestive or lymphatic systems.

Peritoneal mesothelioma is the second-most common form of mesothelioma cancer, accounting for about 15 – 20% of all diagnoses.
Each year, about 500 people are diagnosed with the disease.
Common symptoms include abdominal pain and swelling, weight loss and fluid buildup in the abdominal cavity.
Diagnosis consists of a series of tests, including imaging tests, blood tests and biopsies.
Peritoneal mesothelioma is treated with surgery, heated chemotherapy and immunotherapy.
Peritoneal mesothelioma prognosis may vary depending on an individual’s case, with life expectancy ranging from two to six years.

What Is the Prognosis for Peritoneal Mesothelioma?

The life expectancy for peritoneal malignant mesothelioma cancer is more favorable than other types of malignant mesothelioma.

Peritoneal mesothelioma prognosis may vary depending on an individual’s case, with life expectancy ranging from two to six years. More patients are surviving five years or longer with advancements in treatment.

Survival of Peritoneal Mesothelioma Patients Treated With CRS + HIPEC.

1-year survival - 86%
2-year survival - 77%
3-year survival - 59%
5-year survival - 59%

Several factors can influence an individual patient’s prognosis, including:

Cell type (histopathology).
Tumor size and staging.
Gender and age of the patient.

Epithelioid cell type is most common in peritoneal mesothelioma. It is slower to metastasize (spread) and responds more favorably to treatment, with a median life expectancy of 54 months.

A small percentage of peritoneal malignant mesothelioma patients are diagnosed with sarcomatoid cell type. This type can metastasize quickly and does not respond well to treatment. Peritoneal mesothelioma patients diagnosed with this cell type have an average survival of 4.6 months.

What Are the Symptoms of Peritoneal Mesothelioma?

Peritoneal mesothelioma symptoms can take 10 – 50 years to appear after an individual inhales or ingests asbestos fibers. The fibers become lodged in the peritoneum, the lining of the abdomen, which leads to irritation and scar-tissue buildup. These damaged cells can develop into tumors and cause symptoms.

Peritoneal Mesothelioma Symptoms.

Abdominal pain.
Abdominal swelling.
Anorexia/weight loss.
Fever with no known origin.
Fluid in the peritoneal cavity (peritoneal effusion).
Hypercoagulability (blood clotting).
Inflammatory lesions.
Intestinal obstruction.
Night sweats.

For most patients, early peritoneal mesothelioma symptoms will typically present in the abdomen or gastrointestinal system. Patients may also experience systemic symptoms, such as weight loss, which can further complicate diagnosis.

Resources for Peritoneal Mesothelioma Patients.

How Is Peritoneal Mesothelioma Diagnosed?

A peritoneal mesothelioma diagnosis consists of a series of tests. Diagnosis often starts with imaging tests, like CT scans and X-rays. These tests can rule out more common diseases and other forms of cancer, like adenocarcinoma and ovarian cancer.

If a tumor is detected through imaging tests, a doctor will order blood tests to identify biomarkers that can further differentiate mesothelioma from other cancers.

Biopsies are the only way to confirm a peritoneal mesothelioma diagnosis. A doctor will take a fluid or tissue sample to confirm the cancer. The biopsy can also identify cell type and mesothelioma progression.

Peritoneal Mesothelioma Stages.

When diagnosing peritoneal mesothelioma, a doctor will also determine the stage of the cancer. Although there is no well-defined staging system for peritoneal malignant mesothelioma, doctors may identify the advancement of the disease with relevant criteria. For example, doctors may identify if the cancer has metastasized to other organs or lymph nodes.

In some cases, doctors may also rely on a tool called the Peritoneal Cancer Index (PCI), developed for abdominal cancers. With this method, the abdominal region is split into 13 distinct sections and scored on a scale of 0 – 3 based on the presence and size of tumors.

How To Diagnose Peritoneal Mesothelioma? - What Are the Symptoms of Peritoneal Mesothelioma?
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