Peritoneal Mesothelioma

Peritoneal mesothelioma is a form of cancer that develops in the lining of the abdomen (the peritoneum). It is caused by the ingestion of asbestos fibers, with approximately 500 cases diagnosed annually in the US.

Key Points

  • 1

    Approximately 15 – 20 percent of all mesothelioma cases are peritoneal.

  • 2

    Asbestos likely reaches the abdomen through ingestion or via the lymphatic system.

  • 3

    The average life expectancy of a peritoneal mesothelioma patient is 1 year.

  • 4

    Surgery with a heated chemotherapy wash (HIPEC) is the most effective treatment.

Peritoneal mesothelioma, sometimes called abdominal mesothelioma, is a form of mesothelioma that develops in the lining of the abdominal cavity (known as the peritoneum). It accounts for about 15 – 20 percent of all mesothelioma cases diagnosed throughout the world, with approximately 500 cases in the U.S. diagnosed each year.

Peritoneal form is the second-most common form of the disease (after pleural mesothelioma), and while almost always fatal, it has a more favorable prognosis than other types of mesothelioma. New treatments have extended survival times, with many patients living with the disease for seven years or more.

Free 2018 Peritoneal Mesothelioma Guide

Peritoneal Mesothelioma

  • Cause // Asbestos inhalation or ingestion
  • Location // Abdominal lining (peritoneum)
  • Common Symptoms // Abdominal pain
    Ascites/swelling
    Anorexia/weight loss
  • Treatment // Surgery
    Chemotherapy
    HIPEC Radiation
  • Prognosis // 6 – 12 months

What Causes Peritoneal Mesothelioma?

As with all types of mesothelioma, the peritoneal form is caused by exposure to asbestos. A very small number of people with a specific genetic predisposition may also develop malignant peritoneal mesothelioma after prolonged exposure to erionite, a naturally occurring mineral similar to asbestos.

It’s unclear exactly how asbestos gets into the abdomen, but the two most common theories are lymphatic transport and ingestion.

How Asbestos Reaches the Abdomen
Lymphatic Transport

The lymphatic system is a network of connected tissues and organs critical for helping fight viruses, infection, and disease. Some experts believe asbestos fibers enter initially through the lungs, and then are transported through the lymphatic system to the abdominal cavity, where they can become stuck in the peritoneum.

Ingestion

Asbestos fibers may be swallowed, either directly or after being trapped in mucus during inhalation. The body cannot digest asbestos, and as the fibers move through the digestive system, they may work themselves into the peritoneal cavity and, eventually, into the lining of the abdomen.

Asbestos fibers in the peritoneum can cause inflammation that ultimately leads to cancer. As the tumors grow, they will spread throughout the abdomen and eventually to other parts of the body.

Diagnosing the Symptoms of Peritoneal Mesothelioma

Regardless of the type, mesothelioma can take anywhere from 10 to 50 years to begin to show symptoms. Even when the cancer first starts to present, peritoneal mesothelioma symptoms can easily be confused for other illnesses or diseases because they are nonspecific.

Symptoms of Peritoneal Mesothelioma
  • Breathing difficulties
  • Abdominal swelling due to fluid accumulation
  • Breathing difficulties
  • Fever
  • Anemia
  • Weakness
  • Loss of appetite
  • Weight loss
  • Bowel obstruction
  • Nausea
  • Vomiting
  • Abdominal pain (acute to severe)

Patients experiencing any of these symptoms should be sure to inform their doctor of any potential past exposure to asbestos, even if it occurred many years ago. Malignant mesothelioma and other asbestos-related diseases develop slowly over the course of decades, and no amount of exposure is considered safe.

All forms of mesothelioma are also notoriously difficult to diagnose, leaving some patients with misdiagnoses for months, which ultimately delays treatment. Peritoneal mesothelioma is most commonly mistaken for other abdominal cancers, irritable bowel syndrome (IBS) or ovarian cancer. When facing any cancer diagnosis, it is always a good idea to consider getting a second opinion before beginning treatment. Your primary physician or even general medical oncologists may not have much or any experience with mesothelioma, and could have difficulty properly diagnosing the disease. Scheduling an appointment with a mesothelioma specialist can help ensure you are diagnosed properly and receive the best treatment available.

A diagnosis will typically begin with a variety of tests to rule out other illnesses and types of cancer, including imaging scans, blood tests and biopsies. Currently, a biopsy or tissue sample is the only definitive method to diagnose mesothelioma. The doctor will perform a peritoneoscopy or laparotomy, which is a procedure to take a sample of the tissue from around the abdomen. The biopsy can be uncomfortable, but it is usually over in just a few minutes.

Once the biopsy is taken, a pathologist will scrutinize the sample tissue under a microscope to determine if the cells are cancerous. The doctor will be able to further categorize the cell type and the severity of the abdominal mesothelioma’s progression.

Peritoneal Mesothelioma Staging

Unlike pleural mesothelioma, there is no set staging system for peritoneal mesothelioma because of its rarity. However, doctors may still be able to identify how advanced the disease is and its progression for an individual case. In general, mesothelioma can be staged according to the following characteristics.

The Four Main Stages and Their Characteristics

Stage 1

  • Localized
  • No metastasis or lymph node involvement
  • Curative treatment options
  • Best prognosis

Stage 2

  • Localized
  • Tumor has metastasized with minimal spread to lymph nodes
  • Curative treatment is an option

Stage 3

  • Localized to one side of the body
  • Tumor has spread to nearby organs and lymph nodes
  • Treatment options are usually palliative and non-surgical

Stage 4

  • Spread to both sides of the body
  • Tumor has spread to multiple organs, lymph nodes, and blood vessels
  • Treatment is generally palliative

Peritoneal cancers also have another system that helps identify the severity of tumor progression and helps determine what treatments a patient is eligible for. The Peritoneal Cancer Index, or PCI, is a notable tool that has been used by oncologists for several decades. The PCI works by segmenting the abdominal region into 13 distinct sections. Oncologists will then “score” the region based on the size of the tumors present, known as a lesion score, through imaging scans or laparoscopy.

The overall PCI is then calculated by adding the scores for each region together, with the highest PCI being 39. The regional scores and overall PCI will help oncologists determine if the abdominal cancer is localized or has spread. In general, a higher score means larger, more numerous tumors, indicating a more advanced stage and worse prognosis. Some researchers have proposed the PCI scores can also be translated to the staging system used for pleural mesothelioma.

Peritoneal Cancer Index
Lesion Size (LS) Score and Tumor Size
  • 0: No tumors
  • 1: Up to 0.5 cm
  • 2: Up to 5 cm
  • 3: Larger than 5 cm or multiple tumors
PCI Score and Proposed Mesothelioma Stages
  • 1-10 : Stage 1
  • 11-20: Stage 2
  • 21-30: Stage 3
  • 31-40: Stage 4

Patients with a higher PCI may also be considered ineligible for certain treatments, like surgical removal of the tumors or heated intraperitoneal chemotherapy (HIPEC), which are considered the most effective treatments for peritoneal mesothelioma.

Peritoneal Mesothelioma Prognosis

Mesothelioma in all its forms typically has a rather poor prognosis, which can vary based on a variety of factors including the location and stage. The average life expectancy of peritoneal mesothelioma patients is one year. Thanks to advancements in treatment, patients have started to see an improved life expectancy compared to the other forms of mesothelioma, with even as many as half the patients surviving five years or more.

Peritoneal Mesothelioma Survival Rate
  • 1 year after diagnosis: 92%
  • 3 years after diagnosis: 74%
  • 5 years after diagnosis: 65%
  • 10 years after diagnosis: 39%

The cell type can also have a large impact on the prognosis, as it can help show how aggressive the cancerous cells are and may affect the treatment options available. Epithelioid cells account for 50 – 70% of mesothelioma cases. This cell type is most commonly associated with pleural mesothelioma, but has also been found in peritoneal patients. It’s the most studied cell type and has the best prognosis, though it can be difficult to distinguish from other diseases like adenocarcinoma.

Sarcomatoid is the rarest cell type, accounting for only 10% of cases, and is most commonly found in peritoneal mesothelioma. Sarcomatoid mesothelioma is also difficult to diagnose, and has a higher likelihood of spreading than the other types. The last cell type, biphasic, is a combination of sarcomatoid and epithelial cells. Prognosis for this type will largely depend on the distribution of cells, as the presence of more sarcomatoid cells than epithelial can mean a worse survival rate.

Peritoneal Mesothelioma Prognosis Based on Cell Type
  • Epithelioid mesothelioma: 54 months
  • Sarcomatoid mesothelioma: 4.6 months
  • Biphasic mesothelioma: Not enough data

 

Learn More About Factors That Impact Mesothelioma Life Expectancy

Treatments for Peritoneal Mesothelioma

Mesothelioma specialists often rely on three standard treatments for any type of mesothelioma: surgery, chemotherapy and radiation. Most often, these treatments are used in tandem, known as multimodal treatment, to have the best effect on a patient’s prognosis. Emerging treatments, like immunotherapy, have also been heavily studied in more recent years to determine their effectiveness for mesothelioma. These types of therapies are currently only available in clinical trials, which can sometimes be limited because of mesothelioma’s rarity, but have shown promise in being able to effectively treat the disease and extend overall survival.

For peritoneal mesothelioma patients, there are two types of curative treatment, most often used in combination, that have shown the most positive effects in improving life expectancy. For some peritoneal patients, a minimally-invasive surgery called paracentesis may also be an option for more palliative benefits. The operation removes the fluid build up in the peritoneum, called ascites, to relieve symptoms like breathing issues and improve overall quality of life.

Cytoreductive Surgery and HIPEC

Many studies have shown the positive effects of treating abdominal mesothelioma with cytoreductive surgery in combination with a heated chemotherapy wash called Hyperthermic Intraperitoneal Chemotherapy (HIPEC). The surgery aims to remove all visible tumors in the abdominal cavity, with the HIPEC being applied throughout the abdomen after to kill any remaining cancer cells.

Some studies have found this combination therapy to give patients a median survival of about 53 months or more, with one study even reporting an overall median survival of 92 months with this treatment. Across these studies, about 40 – 50% of patients have achieved at least five years of survival, while overall only 9% of mesothelioma patients typically survive that long.

Peritoneal Mesothelioma Specialists

When facing this rare diagnosis, a mesothelioma specialist at an experienced cancer center will be the best person to evaluate your case and determine the most effective treatment options available for you. Many specialists are also involved in clinical trials and conducting new research to further advance treatment and work towards finding a cure.

Peritoneal Mesothelioma Specialists
Dr. H. Richard Alexander, Jr.
Dr. Richard Alexander, Jr.

Surgical oncologist at University of Maryland Marlene and Stewart Greenebaum Cancer Center

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Dr. Paul H. Sugarbaker

Gastrointestinal surgical oncologist at Washington Cancer Institute

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Dr. James Pingpank

Surgical oncologist at the UPMC Hillman Cancer Center

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Find a Mesothelioma Specialist Near You

Peritoneal Mesothelioma Treatment Costs

The costs associated with peritoneal mesothelioma can grow quickly. According to most recent estimates, the cost of the standard treatment of surgery with HIPEC can average nearly $40,000. Before treatment even begins, medical bills can already add up as diagnostic tests can range from $800 – $2,800. Many patients will also face travel and lodging expenses as many have difficulties finding a mesothelioma specialist close to home.

What patients will have to pay out-of-pocket for treatment will largely depend on their insurance coverage and where they are treated. Even with good coverage, it can still be difficult to keep up with all the bills with any secondary expenses, continued care after treatment, and the loss of income many patients face as they can no longer work.

Since asbestos is the only known cause of mesothelioma, people diagnosed with peritoneal mesothelioma or their surviving family members may be eligible to file a claim and possibly receive compensation to use towards medical costs and lost income. It’s important to speak with a mesothelioma lawyer to learn about your legal rights as soon as possible, as there is a limited statute of limitations in most states.

Rare Forms of Peritoneal Mesothelioma

While peritoneal mesothelioma is rare itself, there are two uncommon subtypes that also have been diagnosed in some instances. Omental mesothelioma and papillary mesothelioma are not well studied or understood because of their extreme rarity, but are still noted as important differential diagnoses.

Omental Mesothelioma

Omental mesothelioma develops in the omentum, a layer of the abdominal membrane that covers organs like the intestines and stomach. It has been noted as a possible result of peritoneal mesothelioma that has spread from the peritoneum, while in other cases it has developed into peritoneal mesothelioma as tumors spread from the omentum. Diagnosis is extremely difficult, and there is no standard treatment because there have been very few cases. As such, researchers have noted omental mesothelioma has a very poor prognosis.

In one of the only cited cases, researchers diagnosed a 54-year old man with omental mesothelioma after he was experiencing severe abdominal pain and they found an indistinct mass in his greater omentum. With further study, they found smaller tumors had spread into the peritoneum, diaphragm and pelvic wall with largely epithelioid cell types. The patient underwent 8 rounds of chemotherapy, and was still doing well 14 months after initial treatment.

Papillary Mesothelioma

Papillary mesothelioma, also called well-differentiated papillary mesothelioma, is a rare variation of peritoneal mesothelioma most often diagnosed in women. The tumors, which are typically benign, usually originate in the peritoneum, though have also occasionally been found in the lining of the lungs and parts of the male reproductive system. Like omental mesothelioma, this can be difficult to diagnose because there are few symptoms and this type of mesothelioma has not been definitively linked to asbestos exposure.

Though there is no standard of care, surgery is usually an option in the documented cases because the tumors typically do not spread elsewhere in the body. In some instances, chemotherapy or radiation have also been applied to shrink the tumors before resection. Studies around papillary mesothelioma have shown strong survival, with patients surviving on average at least 6 years.