Sarcomatoid Mesothelioma



Accounting for 7 to 20 percent of all mesothelioma cases, sarcomatoid mesothelioma is the least common of the disease’s three main histological cell types: Epithelioid, sarcomatoid and biphasic. This type of tumor has also been called sarcomatous, diffuse malignant fibrous and spindled mesothelioma.

These cells appear elongated, spindle-shaped and often form a fibrous pattern that resembles a tumor called histiocytoma. Some epithelioid cells may be present within sarcomatoid tumors, but by definition, they must make up less than 10 percent of the tumor’s mass. Variants of this form of cancer include transitional, lymphohistiocytoid and desmoplastic mesothelioma. Like most other types of the disease, the sarcomatoid cell type is linked to asbestos exposure.


Sarcomatoid Is the Most Aggressive Cell Type

About 10 to 20 percent of pleural mesotheliomas and fewer than 4 percent of peritoneal mesotheliomas are made up of this cell type.

Linked to asbestos exposure.
Tumors appear as nodules or lesions, with or without effusion, and rarely as a localized mass.
More resistant to treatment than other cell types.
Tumors are composed of giant, spindle-shaped cells in fibrous bundles.

and spindled mesothelioma.

These cells appear elongated, spindle-shaped and often form a fibrous pattern that resembles a tumor called histiocytoma. Some epithelioid cells may be present within sarcomatoid tumors, but by definition, they must make up less than 10 percent of the tumor’s mass. Variants of this form of cancer include transitional, lymphohistiocytoid and desmoplastic mesothelioma. Like most other types of the disease, the sarcomatoid cell type is linked to asbestos exposure.

Sarcomatoid Is the Most Aggressive Cell Type
About 10 to 20 percent of pleural mesotheliomas and fewer than 4 percent of peritoneal mesotheliomas are made up of this cell type.

Linked to asbestos exposure.
Tumors appear as nodules or lesions, with or without effusion, and rarely as a localized mass.
More resistant to treatment than other cell types.
Tumors are composed of giant, spindle-shaped cells in fibrous bundles.
QUICK FACT
Most of these tumors develop on the protective lining of the lungs, called the pleura.

Diagnosis

Diagnosing this cell type is difficult because these tumors tend to mimic other benign and malignant conditions in appearance. When pathologists examine these tissue samples under a microscope, the cells resemble those of sarcoma tumors and localized fibrous tumors of the pleura. Because of the aggressive nature of this tumor type, the prognosis is not as favorable as other cell types, and treatment options are not as plentiful. The poor survival rates associated with this cell type make accurate diagnosis even more imperative.

Treatment
As with other cell types, treatment usually depends on the stage and location of cancer. The primary treatment options for this cell type are surgery, chemotherapy and radiation therapy — the same used for other cell types of this cancer.

Surgery
Sarcomatoid cells have proven to be more resistant to treatment than other types of mesothelioma. Surgery, in particular, can be difficult because these tumors are very rigid and often metastasize to the chest wall, making them especially difficult to remove. In cases of peritoneal tumors, cancer usually encases the soft internal organs of the abdomen, which can make the identification and removal of the primary tumor challenging.

Chemotherapy
Chemotherapy has also displayed limited success rates for this cell type. However, a promising drug called selenite has proven to be effective for treating all types of mesothelioma, including this cell type. Researchers found that selenite used in combination with doxorubicin-induced apoptosis (cell death) in sarcomatoid mesothelioma cells. Another combination of chemotherapy drugs that produced encouraging results was CYVADIC, which incorporates cyclophosphamide, vincristine, adriamycin, and dacarbazine.

Features and Diagnostic Techniques
Pathological examination reveals these tumors form nodules that invade surrounding tissues, including the fat found in the parietal pleura. When viewed under a microscope, the tissue contains spindle-shaped cells arranged in a haphazard pattern with plump, elongated nuclei. The same pattern is often seen in fibrosarcoma tumors and thus contributes to misdiagnosis. Sometimes sarcomatoid mesothelioma cells can have more than one nucleus, which makes the tumor easily confusable with fibrous histiocytoma.

To better differentiate mesothelioma from other cancers, pathologists use a tissue staining technique called immunohistochemistry. This technique enhances cell samples with diagnostic markers that react to specific proteins in the tumor tissue. Pathologists can look for these reactions under a microscope to make a diagnosis and determine cancer’s cell type.

A 2015 study explains the most useful markers for diagnosing mesothelioma include cytokeratin 5/6, podoplanin, WT1, and calretinin. The authors of the study add that sarcomatoid tumors may not react to most markers in a majority of cells, however, which makes an accurate diagnosis difficult. They conclude that calretinin and podoplanin are more likely to be effective in diagnosing sarcomatoid mesothelioma, but note a wide variation among laboratories in diagnostic marker choices.

Mesothelioma Variants
Transitional Mesothelioma
This variant behaves like other sarcomatoid mesotheliomas but has plump, spindle-shaped cells.

Lymphohistiocytoid Mesothelioma
Composed of large spindle-shaped cells, lymphohistiocytoid mesothelioma tumors contain a large amount of inflammatory and immune cells mixed throughout. This variant makes up less than 1 percent of all mesotheliomas.

Desmoplastic Mesothelioma
This variant is one of the most difficult cell types to diagnose. At least half of this tumor is “patternless,” and it contains a few elongated cells and collagen bundles. This cell type accounts for approximately 5 percent of all mesotheliomas.

Prognosis
The median survival for patients with sarcomatoid tumors is typically less than six months, but some patients live longer, depending on factors like age and overall health. Patients with the lymphohistiocytoid variant of this cell type have survived for as long as six years, and there are reports of spontaneous tumor remission.

Finding clinical trials designed for sarcomatoid patients may be difficult. Certain specialty cancer centers, like the UCLA Jonsson Comprehensive Cancer Center in Los Angeles, offer programs dedicated to providing more treatment options and expertise than standard cancer care facilities. Alternative therapies and procedures may be considered if traditional treatment options are exhausted.

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