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Peritoneal Mesothelioma cancer forms in the abdomen’s tissue lining, or the peritoneum. It is caused by swallowing or inhaling asbestos. Caused by exposure to asbestos, peritoneal mesothelioma is exceptionally rare, and the prognosis is normally poor.

Peritoneal
Defining Peritoneal Mesothelioma

What is Peritoneal Mesothelioma?

Mesothelioma is a rare form of cancer linked to exposure to asbestos.  Mesothelioma targets the protective membranes of the lungs, heart and stomach and is treated by surgery, chemotherapy and radiation.

There are three types of mesothelioma.  Pleural is the most common form affecting the lining around the lungs.  Peritoneal attacks the lining of the abdomen and is the second most common form.  Pericardial attracts the lining of the heart and is the rarest, affecting only one percent of all mesothelioma diagnoses.

 

Because it is so rare, there has been much less research on pericardial mesothelioma than on the other forms.  There is hope that more treatment options will be developed in the future.

New treatment options often begin as clinical trials.

Chemotherapy and pericardiectomy (surgery to remove the membrane around the heart) can help slow the spread of the cancer although the prognosis is generally six months.  These are difficult treatment choices that require professional guidance.

Peritoneal Mesothelioma Symptoms

Symptoms of Peritoneal Mesothelioma

Because pericardial mesothelioma is extremely rare, it is often not detected until advanced.  It causes pericardial effusion which is a buildup of fluid in the pericardial sac and thickening of the pericardium, preventing normal heart expansion and contraction.

Symptoms include:

  • Heart palpitations
  • Persistent coughing
  • Difficulty breathing
  • Fever
  • Fatigue
Symptoms of Peritoneal Mesothelioma
Peritoneal Mesothelioma Symptoms

What are the Symptoms of Peritoneal Mesothelioma?

Although peritoneal mesothelioma symptoms may resemble symptoms of other less severe conditions such as flu, they could be associated with tumor location near the abdominal cavity.

Common peritoneal mesothelioma symptoms:

  • Abdominal swelling and/or pain
  • Anemia
  • Changes in bowel habit
  • Constipation or diarrhea
  • Fatigue
  • Fever
  • A sense of fullness
  • Nausea or vomiting
  • Night Sweats
  • Unexplained weight loss
Where is Peritoneal Mesothelioma?

Location of Peritoneal Mesothelioma

Peritoneal mesothelioma is a cancer located within the peritoneum, a thin membrane encompassing the abdomen. Caused by exposure to asbestos, peritoneal mesothelioma is exceptionally rare, and the prognosis is normally poor.

Cell Types of Peritoneal Mesothelioma
Peritoneal Mesothelioma Cell Types

Cell Types of Peritoneal Mesothelioma

Tumors of mesothelioma consist of three major cells types that are classified by composition and structure. These three cell types include epithelioid, sarcomatoid, and biphasic.

  • Epithelioid cells respond best to treatments as they are the most common cell type.
  • Sarcomatoid cells respond poorly to treatments as they are the least common cell type.
  • Biphasic cells response to treatments is dependent on the ratio of both epithelioid to sarcomatoid cells.

Some pathologists face difficulties when diagnosing abdominal cancers as there are many rare subtypes of these cells. Additionally – adenoid cystic, bookmark ring, diffuse, microcystic, not otherwise specified (NOS), pleomorphic, tubulopapillary, and well-differentiated papillary are other histological variances of peritoneal mesothelioma.

There is a significant impact on a patient’s prognosis depending on their cell type. For instance, in comparison to patients with sarcomatoid cells, those with epithelioid tumors can typically live an average of 200 days longer.

Examples of rare cell subtypes include:

  • Lymphohistiocytoid Mesothelioma: This is a type of sarcomatoid mesothelioma that is commonly misdiagnosed. A diagnosis of biphasic peritoneal mesothelioma is made when it develops alongside epithelial cells.
  • Desmoplastic Mesothelioma: A sarcomatoid cell type with peritoneal and pleural mesothelioma. The cells are made up of more than 50 percent collagen-producing fibrous tissue.

Deciduoid Mesothelioma: This is a rare variant of epithelial mesothelioma as it has only been diagnosed in around 45 cases. About half of the deciduoids occur in the abdomen.

Peritoneal Mesothelioma Diagnosis

Diagnosis of Peritoneal Mesothelioma

The latest developments in medical technology enable doctors to diagnose this disease earlier than ever. However, it can be difficult for experienced cancer doctors to diagnose it accurately. The process can actually take months.

Doctors have the ability to diagnose this disease earlier than ever due to the latest developments in medical technology. However, it can be difficult for experienced cancer doctors to diagnose it accurately. In fact, this process could take months

The diagnostic process of peritoneal mesothelioma is similar to other types of mesothelioma. It begins with a thorough examination of a patient’s career history, medical history, and overall physical condition. This is generally followed by a number of tests, including image scans and biopsies.

Regardless of if a doctor asks, it is imperative to mention, in depth, any known history of asbestos exposure. This includes the dates of the incidents, locations of the exposure, and the duration and length of the exposure.

This important information alerts doctors of a potential asbestos-related disease and assists in determining the next steps appropriate for a patient. The history of exposure to asbestos is important for directing diagnostic tests as vague abdominal discomfort can be nonspecific.

Quick fact:

It can take up to 20 to 50 years for peritoneal mesothelioma to develop after an individual’s first exposure.

Misdiagnosis of Peritoneal Mesothelioma

Because this cancer is so rare, mesothelioma patients may be misdiagnosed with more common diseases that share similar symptoms by doctors without significant experience. As a result of this serious misstep, patients are delayed vital treatment.

Many symptoms of peritoneal mesothelioma are shared with those that arise with other abdominal cancers or common diseases; these symptoms include abdominal swelling, hernias, and weight loss. The likely hood of a misdiagnosis is significantly increased as a result of symptom overlap.

With a history of asbestos exposure, an appointment with a specialist is the best way to make an accurate diagnosis. Doctors specializing in mesothelioma have the necessary equipment and knowledge to make a quick diagnosis. They can also explain the treatment options which are available to a patient and answer questions or concerns.

Imaging Scans for Peritoneal Mesothelioma

Doctors will likely request a chest x-ray, CT scan, or other forms of imaging scans for a patient that exhibits or experiences symptoms. While potential tumors and cancer spreading are monitored by these tests, they also help doctors to choose the most suitable location for biopsies.

Keep in mind that CT scans can diagnose ascites; fluid in the abdomen. It is extremely important to pursue the cause of ascites in the absence of liver disease.

Biopsies for Peritoneal Mesothelioma

A minor procedure in which fluid and tissue samples are extracted to be microscopically inspected is known as a biopsy. Only a biopsy confirms a diagnosis of peritoneal mesothelioma which makes this step extremely important for the diagnostic process.

In certain cases, a doctor may have to perform an invasive laparoscopic surgical biopsy. For both procedures, pathologists examine whether the results of the laboratory show cancer cells in the peritoneal fluid or tissue.

It is important to test biopsy samples with chemicals referred to as antibodies to confirm a mesothelioma diagnosis. Antibodies, such as calretinin and podoplanin, are used for peritoneal mesothelioma confirmation.

Clinical Trials for Peritoneal Mesothelioma

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.

Finding Peritoneal Mesothelioma Clinical Trials
Stages of Peritoneal Mesothelioma

Peritoneal Mesothelioma Stages

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.

Stage
1

Within Stage 1, Cancerous tissue is minimal and tumors are contained within the abdominal lining, and lymph nodes are free of cancer.

Stage
2

Within Stage 2, Cancerous tissue is moderate and tumors have not spread outside the lining or to lymph nodes.

 

Stage
3

Within Stage 3, Cancerous tissue is more extensive and tumors may have spread outside the peritoneal lining, to lymph nodes or both.

 

Stage
4

Within Stage 4, the mesothelioma has dispersed to numerous locations, such as various other organs and tissues throughout the body. Surgical treatment is no longer an alternative, and most treatments at this stage concentrate on minimizing discomfort and pain.

 

Peritoneal Mesothelioma Treatment Options

Treatment Options for Peritoneal Mesothelioma

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 neighboring 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.

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 anesthesia and surgery.

Surgery for Peritoneal 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 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 cancer.

Tumor spread is usually too vast once it reaches beyond the abdomen for surgery to completely remove cancer. Surgery with curative intent is not recommended after cancer has spread. However, 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 for Peritoneal Mesothelioma

Chemotherapy drugs can shrink peritoneal 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 2017, Dr. Paul Sugarbaker reported improved survival among patients who received early post-operative 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.

Radiation Therapy for Peritoneal Mesothelioma

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 for Peritoneal Mesothelioma

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.

Leading Peritoneal Mesothelioma Physicians
Peritoneal Mesothelioma Doctors

Leading Peritoneal Mesothelioma Physicians

Peritoneal mesothelioma is such a rare cancer that few oncologists have experience treating it. Thankfully, a handful of surgeons and oncologists across the country have chosen to specialize in this type of mesothelioma.

They work at comprehensive cancer centers equipped with cutting-edge technology, and they can get you access to clinical trials. Some of the most renowned peritoneal mesothelioma doctors include:

  • J.F. Pingpack Jr.
  • Paul H. Sugarbaker
  • Charles Conway
  • Suzanne Schiffman
Peritoneal Mesothelioma Life Expectancy

Prognosis of Peritoneal Mesothelioma

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, the 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.

25 percent of peritoneal mesothelioma patients survive three years after diagnosis.

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.

Prognosis of Peritoneal Mesothelioma