Sarcomatoid Mesothelioma

Sarcomatoid mesothelioma accounts for 10% to 20% of all cases, and it is the most treatment-resistant type of mesothelioma. Diagnosis is challenging because the cells can resemble other cancers. Treatment will depend on the cancer's stage and your overall health.

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Patient Advocate Karen Selby explains sarcomatoid mesothelioma.

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This page features: 11 cited research articles

Sarcomatoid: The Most Aggressive Mesothelioma Cell Type

Sarcomatoid mesothelioma is sometimes referred to as sarcomatous, spindle or diffuse malignant fibrous mesothelioma.

Spindle refers to the appearance of the cells when viewed under a microscope. They are long and spindle-shaped.

Diffuse and fibrous describes how the tumors appear within the pleural membrane that surrounds the lungs and lines the chest cavity. Sarcomatoid tumors can merge to form a sheet-like, fibrous mass on the pleural membrane.

It is the least common of all of the types of mesothelioma and also the most aggressive and resistant to treatment.

Nearly all sarcomatoid mesotheliomas are pleural. Fewer than 30 documented cases have occurred in people with peritoneal mesothelioma.

Diagnosis of Sarcomatoid Mesothelioma

Mesothelioma is easily misdiagnosed because signs of the disease mimic other, less serious conditions.

A 2018 study published in Lung Cancer describes a new approach for more accurate sarcomatoid mesothelioma diagnosis. Tests for the BAP1 and MTAP genes were 100% accurate for distinguishing sarcomatoid mesothelioma from fibrous pleuritis.

However, most doctors still use the traditional methods of determining a diagnosis.

Once a doctor has ruled out other causes of ongoing symptoms, they will order imaging studies such as X-rays, a CT scan or an MRI. These tests reveal abnormalities in the chest, but they do not reveal exactly what is causing these changes.

If your imaging studies suggest cancer, your doctor will refer you to a surgeon to retrieve tumor tissue through a biopsy. Surgeons often use thoroscopy or video-assisted thoracic surgery procedures to biopsy mesothelioma. These techniques can increase the chances of an accurate diagnosis.

Seeking a mesothelioma specialty team is important, too, because this group will include pathologists skilled in this diagnosis.

Common Misdiagnoses for Sarcomatoid Mesothelioma

Sarcomatoid cells present an extra level of difficulty to obtaining an accurate diagnosis.

They are easily misdiagnosed as other cancers and some noncancer conditions.

  • Pleural liposarcoma
  • Fibrous pleurisy or pleuritis
  • Sarcoma or fibrosarcoma
  • Malignant fibrous histiocytoma (also called undifferentiated pleomorphic sarcoma)
  • Localized fibrous tumors
  • Metastasized renal cell carcinoma
  • Solitary fibrous tumor of the pleura (hemangiopericytoma)

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Symptoms of Sarcomatoid Mesothelioma

Most patients experience nonspecific symptoms such as cough, shortness of breath and chest pain prior to diagnosis.

As the disease progresses, symptoms become more serious.

  • Shortness of breath
  • Prolonged hoarseness
  • Persistent cough
  • Coughing up blood
  • Low blood oxygen levels
  • Anemia (low iron)
  • Weight loss and weakness
  • Abdominal “fullness”
  • Lack of appetite, anorexia and nausea
  • Fatigue and extreme tiredness

Sarcomatoid Mesothelioma Treatment

A 2019 study published in The Annals of Thoracic Surgery found many patients do not receive treatment consistent with national guidelines on the management of malignant pleural mesothelioma.

The most important way to ensure you receive optimal care is to be treated at a cancer center with experience managing this rare, asbestos-related disease.

The primary treatment options — surgery, chemotherapy and radiation therapy — typically are chosen based on the stage and location of the cancer, regardless of cell type.

Surgery

Surgery is challenging because sarcomatoid tumors are rigid, often spread over large areas and can grow into the chest wall.

All of these features mean surgery alone isn’t likely to be effective for complete tumor removal. in some cases, mesothelioma surgery may not be an option for patients with sarcomatoid disease.

With peritoneal tumors, the cancer can grow into or encase the soft internal organs of the abdomen.

Chemotherapy

The standard chemotherapy for treating pleural mesothelioma is a combination of Alimta (pemetrexed) plus cisplatin or carboplatin. This includes sarcomatoid cell type, which is found almost exclusively in the pleural membrane.

Radiation Therapy

Radiation therapy is not considered curative for mesothelioma. However, it can improve quality and length of life for some patients. It can be used as part of a palliative care plan to reduce symptom severity.

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Prognosis

The median survival for patients with sarcomatoid tumors is typically less than six months, but some patients live longer.

Patients with the lymphohistiocytoid cell subtype have survived as long as six years.

Prognosis estimates are based on past data. As treatment options improve and newer, cutting-edge therapies are employed, prognosis typically improves as well. This effect isn’t captured in current survival rate statistics.

If you are interested in a clinical trial that may improve prognosis, seek treatment at a specialty mesothelioma treatment facility.

Rare Mesothelioma Variants

Transitional Mesothelioma
This variant tends to grow and spread in ways similar to other sarcomatoid mesotheliomas. It has plump spindle-shaped cells, which help pathologists differentiate it from other cells.
Lymphohistiocytoid Mesothelioma
These tumors contains inflammatory and immune cells. Lymphohistiocytoid variant makes up less than 1% of all mesotheliomas. Some patients with this cancer have better-than-average life expectancy.
Desmoplastic Mesothelioma
This variant is a sarcomatoid subtype. It is one of the most difficult to diagnose. Half or more of the tumor shows no discernible pattern of growth. Desmoplastic type accounts for approximately 5% of all mesotheliomas.

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Registered Nurse and Patient Advocate

Karen Selby joined Asbestos.com in 2009. She is a registered nurse with a background in oncology and thoracic surgery and was the regional director of a tissue bank before becoming a Patient Advocate at The Mesothelioma Center. Karen has assisted surgeons with thoracic surgeries such as lung resections, lung transplants, pneumonectomies, pleurectomies and wedge resections. She is also a member of the Academy of Oncology Nurse & Patient Navigators.

Walter Pacheco, Managing Editor at Asbestos.com
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11 Cited Article Sources

  1. Espinoza-Mercado, F. et al. (2019, April 17). Disparities in Compliance with National Guidelines for the Treatment of Malignant Pleural Mesothelioma. DOI:
    Retrieved from: https://doi.org/10.1016/j.athoracsur.2019.03.052
  2. Harris, E.J.A. et al. (2019, January 16). Diagnosis of asbestos-related lung diseases.
    Retrieved from: https://doi.org/10.1080/17476348.2019.1568875
  3. Kinoshita, Y. et al. (2018, November). A combination of MTAP and BAP1 immunohistochemistry is effective for distinguishing sarcomatoid mesothelioma from fibrous pleuritis. :
    Retrieved from: https://doi.org/10.1016/j.lungcan.2018.09.019
  4. Kindler, H. et al. (2018, May 1). Treatment of Malignant Pleural Mesothelioma: American Society of Clinical Oncology Clinical Practice Guideline.
    Retrieved from: http://ascopubs.org/doi/pdf/10.1200/JCO.2017.76.6394
  5. Rossini, M. et al. (2018, April 3). New Perspectives on Diagnosis and Therapy of Malignant Pleural Mesothelioma.
    Retrieved from: https://www.frontiersin.org/articles/10.3389/fonc.2018.00091/full
  6. Husain, A.N. et al. (2018, January). Guidelines for Pathologic Diagnosis of Malignant Mesothelioma 2017 Update of the Consensus Statement From the International Mesothelioma Interest Group.
    Retrieved from: https://www.archivesofpathology.org/doi/pdf/10.5858/arpa.2017-0124-RA
  7. Berg, K.B. and Churg, A. (2017, September). GATA3 Immunohistochemistry for Distinguishing Sarcomatoid and Desmoplastic Mesothelioma From Sarcomatoid Carcinoma of the Lung.
    Retrieved from: https://www.ingentaconnect.com/content/wk/ajsp/2017/00000041/00000009/art00008
  8. Marchevsky, A.M. et al. (2017, September). The differential diagnosis between pleural sarcomatoid mesothelioma and spindle cell/pleomorphic (sarcomatoid) carcinomas of the lung: evidence-based guidelines from the International Mesothelioma Panel and the MESOPATH National Reference Center.
    Retrieved from: https://www.sciencedirect.com/science/article/pii/S0046817717302678
  9. Kinoshita, Y. et al. (2018, November). A combination of MTAP and BAP1 immunohistochemistry is effective for distinguishing sarcomatoid mesothelioma from fibrous pleuritis. 30578-6/fulltext
    Retrieved from: https://www.lungcancerjournal.info/article/S0169-5002(18)
  10. Pavlisko, E.N. and Roggli, V.L. (2015, November). Sarcomatoid Peritoneal Mesothelioma: Clinicopathologic Correlation of 13 Cases. DOI: 10.1097/PAS.0000000000000495
  11. Tischoff, I. et al. Pathohistological diagnosis and differential diagnosis. DOI: 10.1007/978-3-642-10862-4_5
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Last Modified May 6, 2019

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