Desmoplastic mesothelioma is a rare and aggressive subtype of mesothelioma. It is a sarcomatoid mesothelioma with a poor prognosis. Patients can benefit from chemotherapy, immunotherapy, and palliative treatments.
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What Is Desmoplastic Mesothelioma?
Desmoplastic mesothelioma is a rare, aggressive subtype of mesothelioma that mainly occurs in pleural tissue around the lungs. Even rarer are cases of desmoplastic mesothelioma in the peritoneum of the abdomen or the pericardium around the heart.
Desmoplastic tumors are around 50% fibrous and are difficult for doctors to detect. It is considered an aggressive type of cancer. As with other types of asbestos diseases, the earlier it is detected, the better the chances of more favorable outcomes.
How Common Is Desmoplastic Mesothelioma?
Desmoplastic mesothelioma is rare among an already rare cancer. It accounts for around 5% to 10% of all diagnosed mesothelioma cases.
What Are the Characteristics of Desmoplastic Mesothelioma?
All mesothelioma diagnoses can be categorized by primary cell type: epithelioid, sarcomatoid, or biphasic, a mix of the two. Most cases of desmoplastic mesothelioma are sarcomatoid, but some are biphasic.
Sarcomatoid mesothelioma cells are spindle-shaped and resemble the cells of sarcomas, cancers of connective tissue. They readily separate from each other, making it easy to metastasize. All sarcomatoid mesotheliomas are considered aggressive.
Desmoplastic mesothelioma cells form dense, fibrous tissue rich in collagen. They generally lack any pattern, which makes them difficult to identify.
What Causes Desmoplastic Mesothelioma?
Asbestos exposure is the only confirmed cause of any type of mesothelioma. However, some people diagnosed with mesothelioma have no known history of exposure to asbestos.
Case studies of patients with desmoplastic mesothelioma have found that asbestos exposure is a likely primary cause.
Most people with mesothelioma were exposed to asbestos in the workplace years ago. They unknowingly inhaled harmful asbestos fibers that lodged inside the body, triggering inflammation and damage. In some people, this led to cancer decades later.
What Are the Symptoms of Desmoplastic Mesothelioma?
Since desmoplastic mesothelioma tends to occur mainly in the pleural area, it can have the same symptoms as pleural mesothelioma, including:
- Chest congestion
- Coughing
- Fluid buildup in the lungs
- Rib and/or back pain
- Chest pain
- Night sweats
- Fever
- Weakness and fatigue
As with any type of mesothelioma, as the cancer progresses, the symptoms worsen and become more numerous.
Diagnosing Desmoplastic Mesothelioma
Desmoplastic mesothelioma is diagnosed just like any other asbestos-related illness. Physicians perform a biopsy after conducting a physical exam and using imaging scans to find areas of tissue that might be cancerous.
During a biopsy, the doctor removes a sample of the suspected tumor. A pathologist then examines it for cell features.
When diagnosing desmoplastic mesothelioma, doctors look for several characteristics, features, and markers:
- At least half of the tissues are fibrous in nature.
- The tissue is highly collagenized.
- There is necrosis (tissue death) in some areas.
- There are spindle-shaped cells, indicating the sarcomatoid cell type.
- Certain markers are present, including p53, WT1, smooth muscle actin, calretinin, and desmin.
Is Desmoplastic Mesothelioma Ever Misdiagnosed?
Rare mesotheliomas are always difficult to diagnose. Mistaking them for more common illnesses, cancers, and even mesothelioma subtypes is easy.
One condition that is difficult to distinguish from malignant desmoplastic mesothelioma is benign pleural thickening. This is also known as pleural fibrosis, a thickening of fibrous pleural tissue around the lungs that is not malignant.
Benign pleural thickening can result from heart surgery, pleural infections, rheumatoid pleuritis, hemothorax, uremic pleuritis caused by kidney disease, and other non-cancerous conditions.
Some of the characteristics pathologists use to differentiate desmoplastic mesothelioma include:
- A storiform (woven) pattern in the cells
- A general lack of pattern or disorganization in the cells
- Minimal blood vessels
- Invasive growth of the tissue into surrounding tissue
Desmoplastic Mesothelioma Treatment Options
Patients with desmoplastic mesothelioma have similar treatment options to those with other types of sarcomatoid mesothelioma. Because this is an aggressive subtype, it is often advanced when diagnosed. This limits treatment options.
Desmoplastic mesothelioma patients benefit from multimodal treatment. This is a combination of therapies, most commonly surgery, chemotherapy, radiation therapy, and immunotherapy.
Not all patients qualify for surgery if the cancer is widespread. These patients can get some relief from symptoms and extended life expectancy with other therapies, especially chemotherapy and immunotherapy.
A recent study of the immunotherapy drug pembrolizumab (Keytruda) showed positive activity in treating malignant mesothelioma. One patient in the study had desmoplastic mesothelioma and had a 21% reduction in tumor size. Immunotherapy could become a more common treatment for desmoplastic mesothelioma.
Many patients with desmoplastic mesothelioma choose palliative care. Palliative treatments help ease side effects and symptoms, make patients more comfortable, and improve quality of life.
For example, mesothelioma treatments such as chemotherapy and radiation can relieve pressure from the lung area and help reduce tumors.
Another option for patients with rare, aggressive mesothelioma is participating in a clinical trial. This gives them access to new treatments that are not widely available.
What Is the Prognosis for Desmoplastic Mesothelioma?
Desmoplastic mesothelioma has a generally poor prognosis, although every patient is different. The average survival time after a diagnosis of desmoplastic mesothelioma is between 5 and 8 months.
This is a rare subtype, and more information is needed for doctors to formulate a favorable treatment plan that can help extend survival rates.
Studies show that most desmoplastic are in the category of sarcomatoid cancer, which has an extremely low survival rate. Also, most patients are diagnosed in the later stages of mesothelioma, when the cancer is already widespread.
If you received a diagnosis of desmoplastic mesothelioma, talk to your doctors about treatment options. Also, consider talking to an asbestos attorney about your legal rights.
Resources and Help for Asbestos Victims
Remember to fill out our form to get your free Financial Compensation Packet, with information on experienced asbestos and mesothelioma lawyers in your area. Remember, if you’ve been diagnosed with mesothelioma, asbestos-related lung cancer, or asbestosis, you may be eligible for considerable compensation for medical expenses, lost wages, pain, suffering, and much more. For additional assistance or for any pertinent questions, contact us toll-free at 800-793-4540.
