Peritoneal mesothelioma is the second-most-common type of malignant mesothelioma. It develops along the peritoneum, also known as the lining of the abdomen. Unlike the pleural type, patients with this condition survive several years after diagnosis.
Peritoneal mesothelioma accounts for less than 20 percent of all cases of the asbestos-related cancer. Unlike pleural mesothelioma, which forms on the lining of the lungs and is the most common type of the disease, the peritoneal type develops on the lining of the abdomen.
The beginning symptoms of peritoneal mesothelioma, which may show 20 to 50 years after first exposure to asbestos, include abdominal distension, abdominal pain, swelling or tenderness around the abdomen and constipation or diarrhea.
Many of the treatment options for peritoneal mesothelioma are similar to those of other asbestos-related cancers. However, the most promising treatment is a combination of surgery and a heated chemotherapy solution. Patients have credited that treatment to prolonged survival.
The life expectancy of someone diagnosed with peritoneal mesothelioma is usually more than three years, depending on the stage of the cancer, types of treatments the patient undergoes and the quality of the specialist treating the patient.
While 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 that surrounds the abdomen. It has two layers, and peritoneal mesothelioma can develop on both. The parietal layer covers the abdominal cavity, while the 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?
Asbestos exposure is the primary cause of peritoneal mesothelioma, also known as abdominal mesothelioma. Researchers believe asbestos fibers can reach the peritoneum via two pathways:
1.Swallowed asbestos fibers travel from the digestive system to the peritoneum.
2.Inhaled asbestos fibers reach the peritoneum through the lymphatic system, which produces and stores cells that fight disease.
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
Early symptoms of peritoneal mesothelioma can include abdominal pain or swelling, and diarrhea or constipation. Unfortunately, some patients don’t experience any symptoms until tumors spread. This scenario makes treating the disease hard and surviving it even harder.
Common peritoneal mesothelioma symptoms include:
Abdominal pain or swelling
Diarrhea or constipation
Changes in bowel habits
A feeling of fullness
Night sweats or fever
Unexplained weight loss
Nausea or vomiting
Fast Fact: It takes 20-50 years after your first exposure to asbestos for peritoneal mesothelioma to develop.
Once asbestos fibers reach the peritoneum and irritate the cells, the peritoneal lining starts to thicken. The buildup of excess abdominal fluid, known as ascites, may occur next. Once tumors form, they begin to place pressure on the organs.
Diagnosis of Peritoneal 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.
The process of diagnosing peritoneal mesothelioma is similar to other types of the disease. 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, including 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
Because this cancer is so rare, doctors lacking experience with the disease often misdiagnose mesothelioma patients with more common illnesses that share similar symptoms — 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, increasing 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 and other asbestos-related conditions have the knowledge and tools needed to make a prompt diagnosis and explain all the mesothelioma treatment options available to you.
When a patient is experiencing symptoms, such as pain or swelling in the abdomen, 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.
Radiologic biopsies are minor procedures that pull fluid and tissue samples for inspection under a microscope and for other tests. 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.
This delay, combined with the speedy progression of tumors, usually results in a late-stage diagnosis for most patients. Additionally, most patients don’t develop symptoms until the cancer has progressed.
Fast Fact: Testing biopsy samples for antibodies is important for confirming a mesothelioma diagnosis.
Two antibodies used in the diagnosis are BerEP4 and vimentin. BerEP4 is used to diagnose epithelial mesotheliomas. The value of both is under question because they occur in other tumors aside from mesothelioma. Because vimentin develops in other cancers it cannot be used exclusively to diagnose mesothelioma.
Other antibodies used to confirm this disease include cytokeratin 5/6, calretinin, Wilms tumor 1 and epithelial membrane antigen.
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 epithelial, sarcomatoid and biphasic.
Epithelial cells 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 epithelial to sarcomatoid cells.
Because rare subtypes of these cells exist, it presents challenges for pathologists 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 epithelial tumors live an average of 200 days longer than patients with sarcomatoid tumors.
Examples of rare cell subtypes include:
An often misdiagnosed type of sarcomatoid mesothelioma. When it develops alongside epithelial cells, a biphasic peritoneal mesothelioma diagnosis is made.
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.
A rare variant of epithelial mesothelioma. It has been diagnosed in roughly 45 mesothelioma cases. Around half of deciduoid cases develop in the abdomen.
Omental mesothelioma is a rare form of cancer that grows in the omentum, a sheet of fatty tissue in the abdomen that hangs in front of the intestines like an apron. The omentum protects our organs, stores nutrients and prevents the spread of infection. The peritoneum covers the omentum, and both contain mesothelial cells that can turn cancerous and form tumors.
Peritoneal mesothelioma usually causes cancer cells to spread from the peritoneum to the omentum. It’s also possible for cancer to develop in the omentum and spread to the peritoneum, but fewer than five cases exist in medical literature.
How Is Peritoneal Mesothelioma Staged?
Imaging scans help doctors estimate the cancer’s stage. 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.
3Stages of Peritoneal Mesothelioma
Stage I :Cancerous tissue is minimal and tumors are contained within the abdominal lining, and lymph nodes are free of cancer.
Stage II :Cancerous tissue is moderate and tumors have not spread outside the lining or to lymph nodes.
Stage III :Cancerous tissue is more extensive and tumors may have spread outside the peritoneal lining, to lymph nodes or both
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 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 heated intraperitoneal chemotherapy (HIPEC), a treatment that combines surgery and heated chemotherapy.
While doctors only perform HIPEC on a case-by-case basis, it has extended survival and improved quality of life. In studies on small groups of patients treated with HIPEC, around half lived five years or longer.
To qualify for 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 spend more than half of their waking hours up and about and be capable of self-care, which includes basic health maintenance, personal hygiene and the ability to follow doctor recommendations.
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, extend survival and improve quality of life. Palliative therapies do not stop the cancer.
Tumor spread is usually too vast once they reach beyond the abdomen for surgery to completely remove the 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.
Finding Peritoneal Mesothelioma Clinical Trials
Because 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.
Top Peritoneal Mesothelioma Specialists
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
Dr. Paul H. Sugarbaker
Peritoneal Mesothelioma Surgeon and HIPEC Pioneer
Washington Cancer Institute in Washington, DC
Dr. J.F. Pingpank Jr.
Peritoneal Mesothelioma Surgeon
UPMC Hillman Cancer Center in Pittsburgh, PA
Dr. Suzanne Schiffman
Peritoneal Mesothelioma Surgeon
Allegheny Health Network in Pittsburgh, PA
Dr. W. Charles Conway
Peritoneal Mesothelioma Surgeon
Ochsner Medical Center in New Orleans, LA
Prognosis for Peritoneal Mesothelioma
The prognosis for peritoneal mesothelioma varies upon stage at diagnosis, tumor grade, gender and treatments elected. The rate of cancer progression is highly variable in this type of mesothelioma, making it 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.
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.