Desmoplastic Mesothelioma is a rare subtype of Sarcomatoid malignant mesothelioma. It’s an incurable type of cancer that mainly affects the lining membranes of the lungs, abdomen, and heart. The tumors that are associated with this type of cancer are usually more than 50 percent fibrous with a ”bland” and a very hard to recognize pattern. Being a rare type cancer, it only accounts for 5 to 10% of all mesothelioma cases. It’s an aggressive form of cancer that if not diagnosed and treated early enough, it can spread quickly to other body parts/organs. Its prognosis is very poor.
“The term desmoplasia refers to any type of growth of fibrous connective tissue. It is typically a reactive growth in that it begins in response to something else, like a tumor or scar tissue from injuries or surgeries.”
Desmoplastic Mesothelioma Causes
Desmoplastic mesothelioma is associated with prolonged asbestos exposure (inhalation or swallowing of the microscopic fibers/dust of asbestos), radiation treatments to cancers affecting the chest and abdominal region, and certain surgical procedures.
Note: “The cause of formation of the tumor is not well-established.”
Desmoplastic Mesothelioma Diagnosis
Doctors take a large tissue biopsy (a sample of the tumor tissue) so as to accurately diagnose the cancer. A large tissue biopsy is often taken because the fibrous regions of the tumor tend to hide certain cell variations that are crucial to an accurate diagnosis.
However, unlike other types of cancers, the presence of the fibrous tissue in addition to the complex patterns formed by the cells make it a bit challenging for the doctors to diagnose the disease. Sometimes, it’s misdiagnosed as pleural fibrosis, fibrous pleurisy, spindle cell sarcoma or rheumatoid disease. Because of this, doctors are forced to use specific criteria to evaluate when a patient is suspected of being affected by the cancer. The criterion is as follows;
• At least 50% of the tumor must be made up of dense fibrous tissue that tend to form nodules.
• Certain areas of cell death.
• Areas with increased cellularity that have specific sarcomatoid mesothelioma characteristics.
• Metastasis to nearby skeletal muscle, fat tissue or the lungs.
• The spread of neoplastic spindle cells to the chest and lung wall.
Desmoplastic Mesothelioma Symptoms
Majority of desmoplastic mesothelioma cases often go undiagnosed until its late stages since its symptoms are not well-defined in its early stages. Its signs and symptoms usually depend on the location of the tumor.
Tumors located in the pleura or pericardium can cause;
- Shortness of breath, rapid breathing or difficulty breathing.
- Intermittent cough
- Chest pain or discomfort (feelings of fullness or heaviness in the chest).
- Abnormal collection of fluid within with pleural and pericardium.
Tumors in the peritoneum can cause symptoms such as;
- Nausea and vomiting
- Abdominal pain
- Abdominal swelling
- Change in bowel movements.
- Abnormal build-up of fluid in the abdominal cavity (ascites).
Other signs and symptoms include;
- Feeling faint/fainting and dizziness.
- Unexplained weight loss
- Swelling in the lower part of the legs.
- Tiredness or fatigue
- Profuse sweating and low-grade fever.
Desmoplastic Mesothelioma Treatment Options
Because it’s often caught in its late stages, treatment for this type of cancer is usually palliative. This means that it’s mainly focused on alleviating the symptoms, improving patients’ quality of life, and prolonging survival.
Common treatment options include;
- Surgery: Surgery is not usually a curative treatment option, although its helps alleviate symptoms and aids in the diagnosis of the disease.
- Chemotherapy and/or radiation therapy: They are used to shrink tumors and kill the cancerous cells.
- Embolization: This option may be used to provide temporary relief from the symptoms and also minimize loss of blood during surgical procedure.
- Immunotherapy: Immunotherapy boosts the patients’ immune system to kill the cancerous cells.
- Organ transplant: An organ transplant may be an option where the cancer has not metastasized and where the tumor cannot be surgically removed.
- Clinical Trials: Clinical trials may provide patients with new treatments and medications to improve the chances of survival and may be find a cure for the disease. (Learn more about clinical trials).
Desmoplastic Mesothelioma Prognosis
The prognosis for desmoplastic mesothelioma is generally poor since it’s an aggressive form of cancer with invasive malignancy. Although there are various factors affecting the survival rates of the disease such as age, gender, metastasis, histology, and stage of the cancer, the median survival rate is said to be 6 months after diagnosis.