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Desmoplastic Mesothelioma
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Desmoplastic mesothelioma is a rare cell subtype of sarcomatoid malignant mesothelioma. Asbestos exposure is the primary cause. Symptoms include chest pain and fluid buildup in the lungs. Doctors use biopsies to diagnose desmoplastic mesothelioma.
Written by Karen Selby, RN | Medically Reviewed By Dr. Andrea Wolf | Edited By Walter Pacheco | Last Update: July 8, 2024
Written by Karen Selby, RN | Medically Reviewed By Dr. Andrea Wolf | Edited By Walter Pacheco | Last Update: July 8, 2024
What Is Desmoplastic Mesothelioma?
Desmoplastic mesothelioma is a subtype of the sarcomatoid form of mesothelioma. Sarcomatoid refers to long and spindle-shaped cells. It is a rarer and more deadly form of mesothelioma cancer.
Dense fibrous tissue and reduced cellular features characterize the desmoplasia variant. This subtype has a poor prognosis and can occur in all types of sarcomatoid mesothelioma.
Desmoplastic cells are not well-differentiated and lack defining qualities or patterns. These types of mesothelioma cells form dense nodules of connective tissue within tumors. This makes diagnosis more challenging.
This rare variation is present in 5% to 10% of all malignant mesothelioma cases. Asbestos exposure is the disease’s primary cause. It can develop in the different types of mesothelioma cancer.
Diagnosing Desmoplastic Mesothelioma
A biopsy of tumor tissue diagnoses desmoplastic mesothelioma. Doctors take a large tissue sample so they can review enough cells to make a diagnosis. When diagnosing mesothelioma, a sample of tumor tissue is essential. It determines the cell type and treatment options.
Fibrous regions of this tumor can hide cell variations that lead to an accurate diagnosis. Physicians sometimes misdiagnose desmoplastic malignant pleural mesothelioma as fibrous pleurisy. Other misdiagnoses include pleural fibrosis, rheumatoid disease or spindle cell sarcoma.
Desmoplastic Mesothelioma Diagnostic Criteria
- At least 50% of the tumor forms nodules of dense fibrous tissue.
- Areas of increased cellularity are present with sarcomatoid characteristics.
- Neoplastic spindle cells have spread to the lung or chest wall.
- Metastasis has occurred in nearby fat tissue, skeletal muscle or the lung.
- The p53 tumor suppressor gene protein is present.
When desmoplastic mesothelioma spreads to other areas, it can look bland. This causes doctors to mistake the cells as benign fibrous tissue. Imaging scans such as a CT or MRI may help pathologists identify metastasis and diagnose DMM in challenging cases.
In 2017, pathology researchers discovered a protein called GATA-3. It differentiates desmoplastic mesothelioma from sarcomatoid lung cancer. It is present in many mesothelioma cases and often absent in lung carcinoma cases. This factor aids physicians when staging mesothelioma of the pleura.
Symptoms of Desmoplastic Mesothelioma
Dense and fibrous areas may produce symptoms like pulmonary fibrosis, such as chest pain and a buildup of fluid in the lungs. But, these symptoms develop in every case of mesothelioma.
Common Symptoms of Desmoplastic Mesothelioma
- Anemia (low iron)
- Chest pain or abdominal fullness
- Coughing up blood
- Fatigue and extreme tiredness
- Lack of appetite, anorexia and nausea
- Low blood oxygen levels
- Persistent cough
- Prolonged hoarseness
- Shortness of breath
- Weight loss and weakness
The tumor’s cell type doesn’t affect symptoms of mesothelioma. Pleural mesothelioma patients experience shortness of breath. Peritoneal patients often present with abdominal pain.
Most patients don’t know their cell type when I initially speak with them. I help them review their pathology report and explain what sarcomatoid cell types are and what this means for them.
Desmoplastic Mesothelioma Treatment
The treatment options for desmoplastic mesothelioma include immunotherapy, chemotherapy and radiation therapy. These can kill cancerous cells to shrink tumors and limit spreading. Treatment depends on the patient’s age and health.
Immunotherapy with nivolumab (Opdivo) and ipilimumab (Yervoy) is the first-line treatment. Chemotherapy with cisplatin and pemetrexed (Alimta) follows. Radiation can shrink painful chest wall tumors. A 2022 case study including two desmoplastic patients reported that nivolumab (Opdivo) may be more effective for sarcomatoid cell types than epithelial cases because sarcomatoid cells express higher levels of PD-L1 than epithelial cells.
This type of mesothelioma is not treated with surgery. It has a high risk of returning within several months. It is rare for a surgeon to recommend it to desmoplastic patients. A few case reports show some patients lived several months longer following surgery. They were desmoplastic patients with early-stage or localized tumors.
Treatments aim to reduce symptoms, prolong survival and improve quality of life. Doctors recommend pleurodesis or paracentesis to remove fluid from the lungs or abdomen.
Survivor Story
Survivor Story
Wally Rogers
Pleural Mesothelioma
Sarcomatoid Mesothelioma Survivor Treated With Keytruda
After reviewing his records and talking long-distance to the couple, a doctor urged Wally to try Keytruda. “Keytruda is not the answer for everyone,” the doctor told The Mesothelioma Center at Asbestos.com when asked about Rogers. “But here was a chance of him getting a really good result. When it works, the response tends to be long lasting.”
Prognosis of Desmoplastic Mesothelioma
The prognosis of mesothelioma with desmoplasia is not favorable. It resembles other types of sarcomatoid cancer. The disease subtype is tricky to diagnose. Patients often have advanced disease at diagnosis. The life expectancy of patients with this subtype is less than one year.
Sarcomatoid mesothelioma is a little less responsive to chemotherapy. But from what we see from more recent data, it may be more responsive to immunotherapy. And we have additional treatment options for those patients.
Dr. Andrea Wolf, director of the New York Mesothelioma Program at Mount Sinai
A seven-year study evaluated 255 patients with mesothelioma between 1982 and 1989. Researchers identified 17 cases of the desmoplastic subtype. Of those, 11 were sarcomatoid and six were biphasic, comprising both sarcomatoid and epithelial cell types.
The mean survival from the onset of symptoms to death was 5.8 months for the sarcomatoid variant and 6.8 months for the biphasic variant. Other studies show an average life expectancy of 3.8 months.
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