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Peritoneal Mesothelioma | Mesothelioma Master

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A peritoneal mesothelioma is a form of cancer that forms in the peritoneum (the tissue lining of the abdomen). It is caused by inhaling or swallowing asbestos fibers. Patients with this condition may survive several years after diagnosis. Peritoneal mesothelioma is the second-most common type of mesothelioma.

Tumors of this rare asbestos-related cancer form on the abdominal lining.
The beginning symptoms of peritoneal mesothelioma include abdominal distension, abdominal pain, swelling or tenderness around the abdomen and constipation or diarrhoea.

What is Peritoneal Mesothelioma?

Peritoneal mesothelioma accounts for 20 to 25 percent of all mesothelioma cases. It is the most common diagnosis after pleural mesothelioma. Peritoneal patients have longer life expectancies than those with other types of mesothelioma. Some studies report patients living upwards of 5 years after cytoreductive surgery.

Location: Peritoneal mesothelioma occurs in the abdominal lining (peritoneum), a dual-layer membrane that surrounds the stomach and other abdominal organs. The visceral layer protects organs like the liver and gallbladder within the abdomen, while the parietal layer covers the outside of the abdomen.

Peritoneal Mesothelioma

Important Facts About Peritoneal Mesothelioma

  • It accounts for less than 20 percent of all mesothelioma cases.
  • Surgery and heated chemotherapy are the most effective treatments.
  • Immunotherapy for this type is available through clinical trials.
  • Peritoneal patients generally live four times longer than pleural mesothelioma patients.
The survival rate of someone diagnosed with peritoneal mesothelioma depends on the stage of the cancer, types of treatments the patient undergoes and the quality of the specialist treating the patient.
Surgery plays a major role in long-term survival. Patients who don’t qualify for surgery live around one year. About half of patients who undergo surgery and heated chemotherapy are alive more than five years later.

Although clinical trials for peritoneal mesothelioma are not as abundant as those for pleural mesothelioma, researchers are studying whether immunotherapy combined with chemotherapy could play a bigger role in controlling the cancer by boosting the body’s immune system.

Mesothelioma in the Peritoneum

The peritoneum is a protective membrane with two layers that surrounds the abdomen. Peritoneal mesothelioma can develop on both layers.
  • Parietal layer: Covers the abdominal cavity.
  • Visceral layer: Surrounds the stomach, liver and other organs of the abdomen.
Together, the layers support the abdominal cavity and its organs.

What Causes Peritoneal Mesothelioma?

Unlike pleural mesothelioma, peritoneal mesothelioma is most common in females between the ages of 51-59 years old. Men who are diagnosed with peritoneal mesothelioma are often veterans and those with occupational exposure to asbestos. Research suggests that peritoneal mesothelioma patients aged 60 and younger have a better survival rate.

Peritoneal mesothelioma does not usually spread to the lymphatic system (lymph nodes) or bloodstream. It usually metastasizes in large masses in the same areas where it originated.
Asbestos exposure is the primary cause of peritoneal mesothelioma, also known as abdominal mesothelioma.

Development of Peritoneal Mesothelioma

  • Swallowed asbestos fibers travel from the digestive system to the peritoneum.
  • Inhaled asbestos fibers travel from the lymphatic system to the peritoneum.
  • Once fibers are in the peritoneum, they irritate the cells.
  • The irritated cells begin to thicken the peritoneal lining.
  • Excess abdominal fluid builds.
  • Over time, tumors begin to form on the damaged peritoneum.
Research on other causes of abdominal mesothelioma is scarce. Evidence shows other fibrous minerals, such as erionite, and radiation to the abdomen trigger some cases of this disease.

Peritoneal Mesothelioma Symptoms

Due to the location of the disease, peritoneal mesothelioma symptoms most often develop in the abdomen and/or gastrointestinal system, rather than the chest and lungs.
A patient with peritoneal mesothelioma may not experience symptoms early on. If symptoms are evident, they may be mistaken for other illnesses. One common symptom in many peritoneal mesothelioma patients is fluid pockets called ascites, which often cause the stomach region to bulge outward.

The symptoms of peritoneal mesothelioma can take decades to appear after inhalation or ingestion of asbestos fibers. When symptoms do start to appear, they typically develop first in the abdomen and/or gastrointestinal system, though some symptoms (like weight loss or fever) can be systemic.
Symptoms of peritoneal mesothelioma range from those associated with the location of the tumors near the abdominal cavity to symptoms that resemble other less serious conditions such as the flu.

Peritoneal Mesothelioma

Common peritoneal mesothelioma symptoms:

  • Abdominal pain or swelling
  • Diarrhoea or constipation
  • Changes in bowel habits
  • A feeling of fullness
  • Night sweats or fever
  • Unexplained weight loss
  • Nausea or vomiting
  • Fatigue
  • Anaemia
In most cases, peritoneal mesothelioma does not spread to the lungs. It has been shown to spread to the other abdominal areas, such as ovaries, liver, or intestines. This metastasis often causes it to become discovered and sometimes misdiagnosed. Symptoms of stomach pains or ascites sometimes result in a misdiagnosis of hernias or a simple stomachache.

Diagnosis of Mesothelioma

The latest advances in medical technology allow doctors to diagnose this disease earlier than ever before. Yet, experienced cancer doctors can struggle to diagnose it accurately. In fact, the process can take months.

Diagnosing malignant peritoneal mesothelioma is difficult due to non-specific signs and symptoms. It is often confused with abdominal distension (gas) and irritable bowel syndrome. Most patients do not experience symptoms until the disease has progressed. CT scans are the most useful imaging tool to initially test for peritoneal mesothelioma.

Doctors may also use a technique called peritoneoscopy. During this procedure, a surgeon makes a small incision on the patient’s abdomen and uses a small camera to explore the abdomen. There is also a tool on the camera that helps to extract tissue on the peritoneum to test for mesothelioma. These tissue biopsies are needed for confirmation of a diagnosis.

Because the symptoms of peritoneal mesothelioma are often similar to those of other diseases, diagnosis can be difficult. Usually, diagnostic tests focus on first ruling out more common diseases and other forms of cancer.

As part of the mesothelioma diagnosis process, the doctor will determine the stage of the cancer. Although there is no staging system for peritoneal mesothelioma, doctors may try to identify how advanced the disease is using relevant criteria, like if the cancer has spread to the lymph nodes or other organs.

Fluid buildup (ascites) is present in 60-100% of newly diagnosed patients.
Doctors do not use a standard staging system when diagnosing peritoneal mesothelioma. Generally, before the tumors start to spread, the disease is centralized to the abdomen. As it progresses to stage 2, the mesothelioma may spread more but is still contained in the peritoneum. In the final stage, stage 4, the mesothelioma has spread to other organs, such as the liver and colon.

The process of diagnosing peritoneal mesothelioma is like other types of mesothelioma. It starts with a thorough examination of your medical history, occupational history and overall physical condition. A series of tests, including imaging scans and biopsies, usually follows.

Be sure to mention any history of asbestos exposure, even if your doctors forget to ask. It’s important to share every detail you can remember. This includes dates when the incidents occurred and the length and duration of the exposures.

This information will alert your doctors about the possibility of an asbestos-related disease and help them determine the appropriate next steps. Vague abdominal discomfort is very nonspecific, making the history of asbestos exposure important in guiding diagnostic tests.

Misdiagnosis

Because this cancer is so rare, doctors lacking experience with the disease often misdiagnose mesothelioma patients with more common illnesses that share similar symptoms. This is a serious misstep that delays proper treatment.

Peritoneal mesothelioma symptoms, such as abdominal swelling, weight loss and hernias, also arise in other abdominal cancers and many less serious conditions. This overlap of symptoms increases the likelihood of a misdiagnosis.

If you have a history of asbestos exposure, the best way to ensure an accurate diagnosis is to schedule an appointment with a specialist. Doctors who specialize in mesothelioma have the knowledge and tools needed to make a prompt diagnosis. They can explain all the mesothelioma treatment options available to you.

Imaging Scans

When a patient is experiencing symptoms, doctors likely will ask for a chest X-ray, CT scan or another type of imaging scan. These tests will detect potential tumors and show any cancer spread. They also help doctors choose the best biopsy locations.

It’s important to note that a CT scan can diagnose ascites (fluid in the abdomen). In the absence of liver disease, it is extremely important to pursue the cause of ascites.

Biopsies

Biopsies are minor procedures that extract fluid and tissue samples for inspection under a microscope. This is an essential step in the diagnostic process because only a biopsy confirms a peritoneal mesothelioma diagnosis.

Sometimes doctors need to perform a laparoscopic surgical biopsy, which is more invasive. In both cases, pathologists look to see if lab results show cancerous cells in your peritoneal fluid or tissue.
Testing biopsy samples with chemicals called antibodies are important for confirming a mesothelioma diagnosis. Antibodies used to confirm peritoneal mesothelioma include calretinin and podoplanin.

Peritoneal Mesothelioma Cell Types

Mesothelioma tumors are composed of cells classified by their structure and composition. The three major cell types of peritoneal mesothelioma include epithelioid, sarcomatoid and biphasic.
Epithelioid mesotheliomas are the most common and respond best to treatment. Sarcomatoid cells are the least common and respond poorly to treatment. Biphasic tumors are made of epithelial and sarcomatoid cells. The response of biphasic mesothelioma to treatment depends on the ratio of epithelioid to sarcomatoid cells.

Because rare subtypes of these cells exist, pathologists face challenges in diagnosing cancer in the abdomen.

Additional histological variances of peritoneal mesothelioma include adenoid cystic, tubulopapillary, microcystic, signet ring, diffuse, not otherwise specified (NOS), pleomorphic and well-differentiated papillary.

A patient’s cell type can significantly impact their prognosis. Mesothelioma patients with epithelioid tumors live an average of 200 days longer than patients with sarcomatoid tumors.

Examples of rare cell subtypes include:

Lymphohistiocytoid mesothelioma

An often misdiagnosed type of sarcomatoid mesothelioma. When it develops alongside epithelial cells, a biphasic peritoneal mesothelioma diagnosis is made.

Desmoplastic mesothelioma

A type of sarcomatoid cell that occurs in peritoneal and pleural mesothelioma. The cells are composed of more than 50 percent fibrous tissue that produces collagen.

Deciduoid mesothelioma

A rare variant of epithelial mesothelioma. It has been diagnosed in roughly 45 mesothelioma cases. Around half of deciduoid cases develop in the abdomen.

How Is Peritoneal Mesothelioma Staged?

Imaging scans help doctors estimate the stage of the mesothelioma. These tumors initially form on the lining of the abdomen. As the tumors grow and spread, they migrate outside the lining to lymph nodes and distant organs.

For decades, peritoneal mesothelioma experts developed their own staging system because an official one didn’t exist. Since then, researchers have proposed three stages. A fourth stage is not yet clearly defined.
It is generally accepted that patients with extensive tumor spreading are classified as stage IV.

3 Stages of Peritoneal Mesothelioma

Peritoneal Mesothelioma

Stage 1

Cancerous tissue is minimal and tumors are contained 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 to lymph nodes.

Stage 3

Cancerous tissue is more extensive and tumors may have spread outside the peritoneal lining, to lymph nodes or both.

Treatment Options for Peritoneal Mesothelioma

The most effective treatment for peritoneal mesothelioma is cytoreductive surgery followed by Hyperthermic Intraperitoneal Chemotherapy (HIPEC) – a heated chemotherapy “wash” that kills cancer cells within the abdomen.

Although an increasing number of specialists have emerged as treatment leaders for abdominal cancer in recent years, the total number of peritoneal mesothelioma specialists remains small.
If there are no peritoneal malignant mesothelioma specialists nearby, you may consider one in a neighbouring state.
Treatment for this type of mesothelioma includes surgery, chemotherapy and experimental therapies such as immunotherapy.

Doctors believe combining traditional treatments often works better than any single treatment. A combination of one or more treatments is called multimodal therapy. Research shows that a multimodal treatment approach usually offers the best improvement in terms of survival.
The most promising peritoneal mesothelioma treatment is cytoreductive surgery combined with heated intraperitoneal chemotherapy (HIPEC).

While doctors only perform surgery with HIPEC on a case-by-case basis, it has extended survival and improved quality of life. In studies on small groups of patients, around half lived five years or longer.
Treatment options are dependent on the stage a peritoneal mesothelioma patient is diagnosed with. There are more curative treatment options, like cytoreduction surgery, for patients with earlier stages.
Patients in the later stages may receive palliative treatments, like radiation or chemotherapy. Another palliative treatment option may be a paracentesis to drain the fluid buildup in the abdomen.

The most successful treatment of peritoneal mesothelioma has been the combination of cytoreduction surgery and HIPEC (heated intraperitoneal chemotherapy). The cytoreduction removes most of the cancerous tumor, and HIPEC is used to kill the remaining cells.

A study involving Dr. James Pingpank analyzed 211 patients with malignant peritoneal mesothelioma between the years of 1992 and 2010. All of the participants were treated with cytoreduction surgery and HIPEC. Patients that underwent both procedures had a 5-year survival rate of 41% and a 10-year survival rate of 26%. Researchers discovered that the median overall survival of the patients was 38.4 months.
This treatment has been successful in patients with good general health. Some patients have lived for as long as 7 years after having this surgery.

Find a mesothelioma specialist offering this treatment option using our free Doctor Match program.
To qualify for surgery with HIPEC, a patient’s cancer must be limited enough for doctors to completely remove with surgery and not have spread beyond the abdomen.
In addition, qualifying patients must be physically fit in order to tolerate the stress of anaesthesia and surgery.

Surgery

Cytoreduction Surgery

Cytoreduction is also referred to as “debulking.” The goal of cytoreduction is to remove as much of the tumor as possible, though it is often not possible to remove the entire tumor. The peritoneum (lining of the abdomen) and/or some other organs may need to be removed.

Since cytoreduction surgery is such an extensive procedure, it can take a surgeon up to 10 to 12 hours to complete. It is usually performed in patients with stage 1 or 2 peritoneal mesothelioma.
Recovery can take anywhere from 7 to 13 days. One study showed a majority of patients experiencing nausea up to 13 days after their surgery. Regular activities (such as eating, drinking, and mobility) are typically reestablished within 11 days after the cytoreduction and HIPEC.

Peritonal Mesothelioma

Surgery is the most promising traditional treatment option for peritoneal mesothelioma in the abdomen, but it is only effective for early-stage cancer. Because doctors most commonly diagnose asbestos-related diseases after the cancer has spread, most surgeries only attempt to remove sections of the tumor.

Surgery can be curative or palliative. Curative surgery aims to remove as much of the tumor as possible in hopes of curing the cancer. Otherwise, doctors may perform palliative surgery, which aims to remove parts of the tumor to relieve symptoms, including bowel obstruction, extend survival and improve quality of life. Palliative therapies do not stop the cancer.

Tumor spread is usually too vast once it reaches beyond the abdomen for surgery to completely remove cancer. Surgery with a curative intent is not recommended after the cancer has spread. However, a surgery to remove the majority of tumors may be performed to alleviate pain and improve symptoms such as abdominal distention and pain.

Typical surgeries include peritonectomy and cytoreductive surgery, bowel resection and removal of some organs.
Another minor surgical procedure, known as paracentesis, is commonly used on peritoneal mesothelioma patients. A small incision in the abdomen is made to withdraw excess peritoneal fluid, called ascites. This procedure reduces abdominal swelling and pain.

Chemotherapy

Chemotherapy drugs can shrink peritoneal mesothelioma tumors and slow the growth and spread of cancer. It can be given before, during or after surgery. In some cases, doctors offer chemotherapy as the only treatment option. Chemotherapy drugs considered effective against peritoneal mesothelioma include pemetrexed, cisplatin, carboplatin and gemcitabine.

In recent years, a new way of delivering chemotherapy to fight abdominal cancers – including peritoneal mesothelioma, colon cancer, and ovarian cancer, among others – is to use a heated chemotherapy “wash” that rinses out the abdominal cavity and, hopefully, kills any cancer cells left behind after cytoreductive surgery. This technique is called Hyperthermic Intraperitoneal Chemotherapy (HIPEC).

Although HIPEC is performed at only a few cancer centres around the world, it has shown to be more effective than the more common multimodal treatment option of surgery followed by systemic chemotherapy. This may be because the chemotherapy drugs used in heated intraperitoneal chemotherapy treatments are stronger than that used for systemic treatment like cisplatin.
According to a study abstract presented at the 2015 meeting of the American Society of Clinical Oncology (ASCO), approximately two-thirds of peritoneal mesothelioma patients who received HIPEC treatment survived 3.4 years – significantly longer than the standard survival time for peritoneal mesothelioma.

In 2017, Dr. Paul Sugarbaker reported improved survival among patients who received early postoperative chemotherapy and long-term chemotherapy after cytoreductive surgery with HIPEC. All chemotherapy was intraperitoneal chemotherapy, meaning it was applied only to the peritoneum. No systemic chemotherapy was used in the study. Of the 29 patients who had surgery with HIPEC, post-operative chemotherapy and long-term chemotherapy, 75 percent lived longer than five years.
In appropriate patients, the main treatment will be cytoreductive surgery with HIPEC. The surgical part removes all gross tumors with residual deposits smaller than 2 mm. The HIPEC treats the residual tumor and microscopic cancer cells the surgeon can’t see.

Peritoneal mesothelioma patients may be given a combination of Alimta and cisplatin, the same treatment given to pleural patients.
In 2009, a patient went in for an unrelated procedure and signs of peritoneal mesothelioma were found. The patient was given a combination of Alimta (500 mg) and cisplatin (80 mg).
After six cycles of chemotherapy, all signs and markers of the peritoneal mesothelioma were gone. Six months later, CT scans showed no changes or reoccurrence regarding the mesothelioma. After 4 years, the patient was alive with no signs of disease progression.

Studies show the normal survival rate of patients with peritoneal mesothelioma is around 7.6 months if they do not receive chemotherapy. Forty-one percent of patients who were given the combination experienced improvement, while 17% showed satisfactory results with cisplatin alone.
On average, patients who received the combination of Alimta and cisplatin lived 12.1 months longer compared to 9.3 months on just cisplatin.
Studies are also testing the effectiveness of the drugs vinorelbine and gemcitabine in combination with cisplatin.

Radiation Therapy

For many peritoneal mesothelioma patients, radiation is not usually effective. Radiation may shrink tumors before or after a cytoreduction, but there have been no reported cases of eradicated mesothelioma by solely using radiation. A mesothelioma specialist can determine if radiation is appropriate based on their patient’s specific diagnosis.

Some studies show radiation therapy can improve peritoneal mesothelioma survival slightly when combined with surgery and chemotherapy. However, doctors tend not to recommend radiation for these patients.
Even though targeted radiation can shrink tumors and slow cancer growth, the procedure is risky because of the location of these tumors. The peritoneum wraps around the stomach, liver and intestines. Aiming radiation at nearby tumors could harm these organs and cause life-threatening damage.

Alternative Treatments

Alternative treatments and emerging therapies are available, but these treatments have less predictable outcomes. Immunotherapy boosts the body’s immune system to help fight cancer. Research on this emerging therapy mainly focuses on the treatment of pleural mesothelioma, which develops in the lining of the lungs.
Many patients find hope in clinical trials, which are medical studies that test new and experimental treatments. Research from clinical trials helps improve treatment outcomes for future patients.

Finding Peritoneal Mesothelioma Clinical Trials

The number of new peritoneal mesothelioma cases is low. About 500 patients are diagnosed each year in the U.S. The number of people in clinical trials for this cancer is even smaller.
You can talk to your doctor or a patient advocate at The Mesothelioma Center about whether a mesothelioma clinical trial is right for you.

A recent example of a peritoneal mesothelioma clinical trial is the drug tremelimumab. The study recruited patients to test tremelimumab’s effectiveness against mesothelioma. It’s an immunotherapy drug that signals the immune system to attack malignant mesothelioma cells. The study paired tremelimumab with chemotherapy to increase effectiveness.

In 2017, Ochsner Medical Center in New Orleans opened a clinical trial to study the effects of a new immunotherapy drug in combination with chemotherapy for peritoneal mesothelioma.

Prognosis for Peritoneal Mesothelioma

While the prognosis for all forms of mesothelioma is generally poor, the life expectancy for peritoneal mesothelioma is somewhat better than for other types of malignant mesothelioma. The median survival period for abdominal mesothelioma is 1 year, and the longest known survival was 19 years.
Patients who undergo cytoreductive surgery with HIPEC have significantly better survival rates. According to one study, approximately half of peritoneal mesothelioma patients who undergo this form of treatment lived more than five years.

The prognosis for peritoneal mesothelioma depends on:

  • Stage at diagnosis
  • Tumor grade (how fast it grows)
  • Gender
  • Genetic mutations
  • Treatments selected
The rate of cancer progression is highly variable in this type of mesothelioma. It is hard to predict individual prognosis.
In the ’80s and ’90s, average survival time was around one year. Today, more people are living longer with this disease. Survival is significantly longer for patients who qualify for surgery with heated chemotherapy. Nearly half of these patients live longer than five years.

More than 60 percent of patients are diagnosed too late to qualify for surgery. These patients often elect chemotherapy. The combination of cisplatin and pemetrexed delivered systemically has a response rate around 30 percent with average progression-free survival around 11.5 months and median survival around 13 months.

Chemotherapy delivered directly, not systemically, to the peritoneum without surgery has a higher response rate of 47 percent. Meanwhile, heated chemotherapy delivered during surgery has a response rate of 84.6 percent.

Women tend to live longer with peritoneal mesothelioma than men. When short- and long-term survival is averaged out, women live an average of 13 months, and men live six months.
The median survival of untreated peritoneal mesothelioma is six months.
Patients with tumors containing epithelial cells live longer than patients with sarcomatoid or biphasic cells. The tumor’s grade also impacts prognosis. Tumor grade is based upon how abnormal the cells appear, which indicates how quickly they are likely to grow and spread.

Although there isn’t currently a cure for peritoneal mesothelioma, many patients have a hopeful prognosis. The median survival time for patients who have not had the cytoreductive surgery is about a year; however, in patients who have had the surgery, survival times increase by up to five years. There have been cases of long-term survivors who have been in remission for over fifteen years. The most successful cases are those whose mesothelioma is detected in the earlier stages and begin treatment immediately. Most of these cases include a cytoreductive surgery and HIPEC.

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Mesothelioma Master: Peritoneal Mesothelioma | Mesothelioma Master
Peritoneal Mesothelioma | Mesothelioma Master
Peritoneal mesothelioma is a cancer found in the peritoneum.Surgery and chemotherapy are the most successful treatments to extend life expectancy.
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