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Epithelioid Mesothelioma | Mesothelioma Master

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Epithelioid mesothelioma is a cancer caused by epithelial cell mutations related to asbestos exposure. The epithelioid cell type accounts for about 70 percent of mesothelioma cases. It has a better prognosis than other cell types because it is less aggressive. There are three types of mesothelioma cells: epithelioid, sarcomatoid, and biphasic. Epithelioid mesothelioma is not only the most common. But it is also the most studied and has the best prognosis.

What is Epithelioid Mesothelioma?

Epithelial cells in the body are very common and they are also healthy. They become dangerous when they mutate themselves into deadly epithelioid mesothelioma cells after being exposed to asbestos. Individuals with epithelioid mesothelioma have a better prognosis. And more treatment options available to them vs. those that have sarcomatoid or biphasic cell types. Cancer is the mutation of a previously healthy cell. Cells are programmed to perform a certain duty, to divide. And when they become old, they are programmed to die. Genetic changes to normal cells allow them to ignore their programmed death and continue to divide.

Epithelial cells are one of the four tissue types found in the body. They form the lining for organs, hollow cavities, and our skin. When they are exposed to a carcinogen (like asbestos) it causes genetic changes that turn these cells cancerous. Approximately 50 – 70 percent of all mesothelioma cases are epithelioid. Which means it’s the one most studied and has the best prognosis.
Epithelial pleural mesothelioma refers to a specific type of mesothelioma. That affects the protective tissue surrounding the lungs, called the pleura. And where epithelial cells are present, which look like small, square cells with a tubular configuration and visible cell nucleus. When these cells become cancerous, they are referred to as epithelioid cells. Epithelial cells themselves are not cancerous, but rather mutate into a cancerous form. The mesothelium (the membrane that lines different cavities in the body) is made up of epithelial cells. And when such mutations take place, mesothelioma of the epithelioid cell type develops.

When looking at the cause of epithelial mesothelioma, asbestos exposure is the only known cause. Though there are a variety of risk factors that are emerging as possible contributors to the development of the disease. Mesothelioma develops in the mesothelium, a lining made of epithelial cells. When epithelial cells of the mesothelium turn cancerous, they alter in appearance. And take on patterns called epithelioid, sarcomatoid or biphasic. Epithelioid mesothelioma, which is also called epithelial mesothelioma, is the most common type of the asbestos-related disease.

Epithelioid Mesothelioma

How Is It Diagnosed?

Epithelioid mesothelioma is difficult to distinguish from certain other forms of cancer, like adenocarcinoma. Adenocarcinoma is formed from glandular epithelial cells. It is the most common form of lung cancer, but it can occur wherever mucus-producing epithelial cells reside.
It’s important for physicians to rule adenocarcinoma out prior to treatment. As the treatment protocols are different from mesothelioma.

Recently, researchers have discovered various stains that. When applied to the cells and then viewed under a microscope, reveal the type of cancer the patient has. Research continues to be done to further reduce the chances of an incorrect diagnosis. Epithelioid cells cannot be identified with diagnostic imaging scans. To determine which cell type is present. Doctors need to perform a thoracoscopy or similar form of surgical biopsy. Biopsies offer doctors a way to examine the potentially cancerous cells under a high-powered microscope. During a biopsy, a tissue sample of the tumor is extracted for further evaluation of the cells it contains.

Diagnosing a patient's mesothelioma cell type is the most important stage of the diagnostic process. Knowing a patient's cell type helps doctors determine the best course of treatment.
After surgeons take a biopsy of the diseased tissue, they send the sample to a pathologist. General oncologists and surgeons can make guess the diagnosis based on a patient's symptoms. But only pathologists can confirm the diagnosis and cell type. Pathologists study tissue samples under a microscope, looking for the defining characteristics of the cancerous cells.

Where It Occurs

Epithelial mesothelioma most commonly occurs in the pleural cavity – the area around lungs. In fact, 70 percent of all cases of pleural mesothelioma are epithelioid. The other common locations for mesothelioma (the abdomen, and very rarely, the lining of the heart) are more commonly sarcomatoid or biphasic.

Epithelioid pleural mesothelioma can be difficult to diagnose. Patients often first visit their primary care physician with complaints of chest pain or shortness of breath. Referral to a pulmonologist is common before an oncologist (cancer doctor) becomes involved.
Diagnosing pleural mesothelioma quickly can be problematic because symptoms don’t become obvious until the cancer progresses. These symptoms also often mimic less severe respiratory conditions. Such as asthma or pneumonia, which may lead to an initial misdiagnosis.

Once the symptoms of pleural mesothelioma are recognized, additional diagnostic tests like imaging scans. And testing tumor samples are conducted to confirm the presence of cancer. Detecting epithelioid cells requires an experienced pathologist. Who analyzes tumor samples to determine which cell type is present. There are subtypes of epithelial mesothelioma that an experienced pathologist knows how to test for. And they know how to tell these cells apart from cells of other types of cancer.
Because epithelial pleural mesothelioma can resemble adenocarcinoma cells. It’s important to have an experienced pathologist analyzing the tumor samples.

How It Develops

Epithelioid mesothelioma develops when epithelial cells mutate into cancerous cells – that is. Those cells that no longer serve their original purpose and have uncontrolled division. With only the rarest exception, asbestos exposure causes the mutation.
The asbestos type that is most likely to cause mesothelioma has tiny, needle-like fibers that pierce the lungs when inhaled. Over time they work their way through the inside of the lung to the outer lining. This causes inflammation and irritation in the lining of the pleural cavity. Which is composed of epithelial cells. Gradually the epithelial cells undergo genetic mutation and become cancerous.
Occasionally asbestos can work its way into other organs and cause mesothelioma in different locations. But this is not common.

Different characteristics help pathologists differentiate between the subtypes of epithelial mesothelioma. For example, tubulopapillary epithelioid mesothelioma cells form a cube-like shape. Histiocytoid cells resemble pulmonary macrophages, and poorly differentiated cells are round or irregularly shaped.

Though statistics on all the epithelioid subtypes aren’t available. It is known that deciduoid epithelioid mesothelioma accounts for approximately 2-5 percent of all mesothelioma cases.  And small cell accounts for less than 6 percent. Most epithelial cases have a tubulopapillary cell pattern, while rare cases are adenoid cystic or signet ring. A mesothelioma diagnosis can be extremely jarring. However, the sooner that the cancer is diagnosed, the better the treatment options and life expectancy for the patient. Misdiagnosis and a long latency period often prevent early detection, so whenever symptoms of mesothelioma are recognized. It’s crucial that the individual seek medical attention as soon as possible and consult with a mesothelioma specialist.
One of the primary challenges of diagnosing epithelioid mesothelioma is distinguishing it from other types of cancer. Cancer in epithelial tissue could be a number of malignancies, which is why extensive testing is important. Epithelioid mesothelioma is often confused with adenocarcinoma. A common type of cancer that develops in the lungs, breasts and colon. Glandular mesothelioma, an epithelial cell subtype, may resemble adenocarcinoma of the lungs. It may be difficult to differentiate these two conditions.

What Is the Best Epithelioid Mesothelioma Treatment?

Epithelioid mesothelioma responds better than sarcomatoid or biphasic mesothelioma to treatment. It is less aggressive and metastasizes slower than other cell types. This means surgery is more effective for epithelioid patients because their cancer cells don't spread as quickly. Depending on the stage of a patient’s cancer, there are aggressive courses of treatment that may improve a patient’s prognosis.
Treatment for mesothelioma cancer typically depends on the type and stage of cancer, rather than the cell type. This means that treatment for epithelial pleural mesothelioma is similar to the treatment of other mesothelioma cell types. Chemotherapy, radiation therapy and surgery may be viable options for epithelial pleural mesothelioma patients.

Because epithelial cells have proven to respond better to treatment, epithelial pleural mesothelioma patients may be eligible for a more aggressive treatment plan. And maybe given a better prognosis than patients with other cell types. Biopsies are one of the first steps in defining a general treatment plan. For example, if epithelioid malignant mesothelioma is caught in the early stages, surgery. And chemotherapy is likely options for treatment. However, late-stage diagnoses might leave patients confined to palliative care, as they may be too weak to handle treatment side effects.

Since epithelioid mesothelioma cells respond best to treatment. A patient with this type may be considered for a more aggressive treatment plan. Epithelioid patients diagnosed before the cancer has spread throughout the chest often qualify for multimodal therapy. Which attempts to kill cancer cells using multiple types or modes of therapy. The multimodal therapy combines the most effective anticancer treatments for mesothelioma, including surgery, chemotherapy and radiation therapy.
In 1999, Dr. David Sugarbaker published impressive survival results among a group of pleural mesothelioma. Patients with malignant epithelial cells who had the multimodal therapy that included an extrapleural pneumonectomy surgery, chemotherapy and radiation therapy. About 46 percent of patients who had epithelial cell type. No lymph node involvement and no remaining cancer cells after surgery lived at least five years. The typical five-year survival rate for mesothelioma cancer is around 10 percent.

Less than half of epithelioid patients qualify for aggressive surgery and multimodal therapy. And more than half are diagnosed too late to qualify for surgery regardless of cell type. When surgery isn’t an option, chemotherapy is considered standard of care and clinical trials are discussed.

Common Mesothelioma Treatment Options

For epithelioid malignant mesothelioma, the most common treatment plan is a combination of surgery. Chemotherapy and radiation, typically used together in a technique referred to as a multimodal treatment. Since mesothelioma currently has no cure. The main goal is to eliminate or alleviate symptoms and provide the patient with the longest life expectancy possible.

Surgery

For patients who are in otherwise good health, this potentially curative surgery removes the entire lung, mesothelium (lung lining). Half the diaphragm, and pericardium (heart lining) on that side. This is a major surgery with a high rate of complications, but if the patient meets the requirements. Some researchers argue it can potentially cure the disease.
  • Used curatively or palliatively
  • Targeted treatment
  • Meant to remove as much of the cancer as possible, or reduce pain and discomfort. Such as by removing fluid buildup that can put pressure on affected organs

Chemotherapy

This therapy can reduce the size or decrease the advancement of mesotheliomas. But this positive response does not last.
These drugs attack what makes cancer cells different than healthy cells. This relies on understanding the particularities of that cancer, as the genetic changes between two patients with the same cancer may differ.
  • Used curatively or palliatively
  • Addresses mesothelioma cells throughout the body
  • Meant to destroy cancer cells and shrink tumors, alleviating associated symptoms

Radiation Therapy

Radiation therapy is not usually very effective in mesothelioma because the tumor is rarely contained in one area. This makes it difficult or impossible to concentrate the radiation waves at only the affected tissue.
  • Targeted treatment
  • Aims to kill cancer cells
  • Known to help with pain and shortness of breath
When epithelioid mesothelioma is treated you may undergo the same treatment that you would with other types of mesothelioma. The good news is that this treatment will be a lot more effective than treatment used with other types of mesothelioma cancer.
There are a number of patients that are able to receive extrapleural pneumonectomy and it has been found to increase your life expectancy. You may be able to go through with pleurectomy with decortication (P/D) if the cancer has not made its way to the lung.

If you have epithelioid peritoneal mesothelioma then you might want to think about cytoreductive surgery. There are no stages with peritoneal mesothelioma. Therefore, it is always best to work with a specialist and get a second opinion.
Other treatment options such as chemotherapy and even radiation may be used. Alternative treatment like gene therapy or even intensity modulated radiation have been found to be effective.

Prognosis of Epithelioid Mesothelioma Is Good

The best prognosis is associated with mesothelioma composed of epithelioid cells. The median survival time with epithelioid mesothelioma is about one year after diagnosis. Comparatively, patients with sarcomatoid mesothelioma live an average of six months.
The improved prognosis is around 200 days on average, but it could amount to years. If the cancer is diagnosed in an early stage.

In 1996, a now well-known Swedish study examined tumor cell type as a prognostic factor in 85 cases of pleural mesothelioma. Patients with epithelioid mesothelioma survived about 200 days longer than patients with sarcomatoid or biphasic cell types. Those with tubulopapillary cells, a subtype of epithelial mesothelioma. Lived 275 days longer than patients with other cell types.
Overall, epithelioid mesothelioma is associated with better response to treatment and longer survival. This cell type can open a window for patients to access aggressive treatment plans and innovative clinical trials.

Cancer stages are based on the TNM classification, which rates the patient’s cancer on three factors: Tumor, Lymph Nodes, and Distant Metastasis. Essentially, researchers and physicians want to know to what extent the tumor has formed. If it has travelled, how far and how deeply embedded in other tissues has it become. Patients are told their cancer falls into a “stage” of 1 through 4. These stages are a cumulative assessment of the TNM classification.
One study broke down the difference in prognosis and survival between patients with epithelioid mesothelioma. All of these patients received surgery and a mix of chemotherapy and radiation therapy.
  • Patients with epithelioid mesothelioma had a 2-year survival rate of 50 percent, and a 3-year survival rate of 42 percent.
  • Patients with the rarer sarcomatoid mesothelioma had a 2-year survival rate of 7.5 percent, and none lived past 25 months.
The median survival time for an epithelioid mesothelioma patient is 12 to 24 months. Compared with 12 months for biphasic patients and 6 months for sarcomatoid patients. As with diagnosis and treatment plans, the prognosis varies from patient to patient and depends on a variety of factors. In terms of general prognosis, those that are undergoing multimodal care are likely to experience a better prognosis. And longer life expectancy, compared to those that result solely in palliative treatments. Drug combination therapy has also positively impacted survival rates with pemetrexed and cisplatin showing much promise when used together.

Cell type is a very important factor that can have a huge impact on a patient’s mesothelioma prognosis. Those with epithelioid mesothelioma have a better prognosis than those with biphasic or sarcomatoid cell types.
There was a study that showed that roughly 60% of those patients that have been diagnosed with epithelioid mesothelioma. And treated for it, lived for at least one year after they started their treatment. Approximately 25% were found to live for more than 5 years. 

Epithelioid Mesothelioma Life Expectancy

The median survival rate for those diagnosed with epithelioid mesothelioma is 18 – 24 months. Compared to sarcomatoid mesothelioma, treatment options have a much more positive impact on life expectancy. With 60% of epithelioid patents showing survival rates of over a year after receiving treatment. This is in part because the epithelioid type is much less aggressive than sarcomatoid and responds better to treatment.

Nuclear grading is used by many as a way to pinpoint life expectancy. Epithelial cells have a well-defined nucleus, making them an excellent contender for the grading system. For those with a nuclear grade of 1, the median patient survival is 28 months. Increased grades lead to lower survival rates, with grade 2 averaging to 14 months and grade 3 averaging 5 months.
Although life expectancy for all malignant mesothelioma types is bleak. Research and emerging treatments continue to establish hope for improving the lives of those diagnosed. And offering more options to extend survival rates and contribute towards finding a cure.

What Are the Characteristics of Epithelioid Cells?

Tumors are classified by the type and appearance of the cells involved. Normal epithelial cells from the epithelium, which is the most common of the four major tissue types in humans. Those types include epithelial, connective, muscular and nervous tissue. With functions including protection, sensory perception and fluid secretion. Epithelial tissue lines several major body cavities and most of our organs. Epithelial cells are also present in our skin, eyes, taste buds and ears.
The structure of epithelial tissue will vary depending on its location and function. Epithelial cells may appear thin and flat and shaped like cubes, hexagons or columns. When the epithelial cells turn cancerous, they can take on several visual patterns: Epithelioid, sarcomatoid and biphasic. Usually, they lose uniformity or otherwise become atypical in appearance, but they can also form small tubes or clusters that resemble a raspberry.

Cancerous cells are often differentiated based on how they grow and spread, along with their shape and size. Epithelial cells aren’t as fast-spreading as sarcomatoid cells with their uniform formations, but they are known to spread locally and to the lymph nodes.
In terms of location, epithelioid mesothelioma is most commonly pleural-based, located in the linings of the lung. But can also be found in the abdomen, genitals and other reproductive regions.
When looking at cell shape, epithelial cells stand apart with a well-defined nucleus that can be used as a determining factor in cell type during biopsies. Typically epithelioid mesothelioma cells are flat or cube-shaped, but when they become cancerous they can take a variety of shapes, depending on subtype.

Epithelioid Mesothelioma

Cell Prevalence

Epithelioid mesothelioma is the most common cell type and it also has the best prognosis. You will find that it is the most common with men that are white and older than 45 years of age. They are square-shaped cells and have visible nuclei (plural for "nucleus," the centre of the cell, which carries genetic material). Specialists have an easier time telling them apart from other mesothelioma cell types because of their unique appearance.

Cell Description

These cell types have a defined elongated egg-shape to them. The visible nuclei make this cell the easiest type to distinguish.
Tumors made of epithelioid cells grow quickly. These cells replicate faster than sarcomatoid or biphasic mesothelioma tumors. However, the square shape of epithelioid cells causes them to stick together, slowing down their spread to other parts of the body.

Cell Behavior

A tumor with these cells will grow much faster because they are going to divide very fast. They stick to each other which means it doesn’t spread as fast.
Epithelioid mesothelioma responds the best to treatment because it metastasizes (spreads) slower than other cell types.
Because epithelioid cells lack mobility and adhere closely together, they are less likely to spread, as sarcomatoid cells do, to distant locations. Epithelioid cells primarily spread to nearby lymph nodes and from there migrate locally via the lymphatic system. Conversely, sarcomatoid cells are loosely organized, and they can migrate easily, leading to quicker metastasis.

Epithelioid cells are more common in cases of malignant pleural mesothelioma than peritoneal mesothelioma. A certain type of epithelioid mesothelioma occurs more commonly in women, and it’s known as well-differentiated papillary mesothelioma. No other cell type is associated with a particular gender, age or race.

Epithelial Mesothelioma Subtypes

Under epithelioid mesothelioma, there are several subtypes that vary based on cellular structure, location and symptoms. The subtype can affect side effects, treatment and prognosis for the mesothelioma patient. There are a number of epithelioid mesothelioma subtypes. They are going to have variations related to their sizes, shapes, and even their structure. Each subtype may respond differently to different types of treatment.
Epithelioid cells come in different shapes and sizes. Each cellular subtype responds differently to treatment. Some subtypes are more common than others, making them slightly easier to diagnose and treat.

Epithelioid mesothelioma has many subtypes, each with its own unique characteristics. Some subtypes are more likely to develop in specific parts of the body, while others are extremely rare. While the subtype you have won’t affect your treatment, it does help doctors tell mesothelioma apart from similar cancers.
The following are technical descriptions of some cell patterns doctors have observed in cases of epithelial mesothelioma.

Tubulopapillary

The tubulopapillary cell pattern is one of the most common subtypes of epithelioid mesothelioma. Most tubulopapillary mesotheliomas contain well-differentiated cells. Doctors may mistake this subtype for adenocarcinoma that has spread to the pleura.

Adenomatoid

Otherwise known as glandular or microglandular mesothelioma, these cells can be flat or cube-like and can line glands. Which can make it difficult to differentiate from adenocarcinoma.
Adenoid epithelioid cells are found in all types of mesothelioma. Because it does look like other tumors it can be hard to diagnosis a patient accurately.
Adenomatoid mesothelioma, also known as the microglandular subtype. Accounts for 6 percent of all pleural mesothelioma cases. These tumors are made of bland cells that are flat to cube-like in shape and lined by small gland-like structures.

They often appear alongside other subtypes but may also be the dominant cell pattern. It can be difficult for doctors to tell this subtype apart from other tumors. Including benign adenomatoid tumors and metastatic adenocarcinoma of the pleura.

Solid

The solid subtype has two patterns: Well-differentiated and poorly differentiated.
Solid well-differentiated is one of the most common cell patterns seen in mesothelioma cancer. Its round cells form nests, cords or sheets. The poorly differentiated pattern has relatively unorganized cells that are polygonal to round in appearance.
Solid well-differentiated mesothelioma may be mistaken for benign reactive mesothelial hyperplasia. While the poorly-differentiated pattern appears similar to lymphoma and large cell carcinoma.

Small Cell Mesothelioma

Small cell mesothelioma has its own shape. You will see that it is the most common when you look at peritoneal mesothelioma. It will be the most difficult to treat.
This very rare form of epithelioid mesothelioma was first discovered in 1992. It looks very similar to other small cell forms of cancer like small cell lung carcinoma. Pathologists diagnose small cell mesothelioma through cytologic analysis (looking at the cells through magnification).

Glandular

Tumors with the glandular pattern are mostly composed of acinar or gland-like structures. This subtype usually develops in the pleural lining. It may be confused for adenocarcinoma that has spread to the pleura.

Cystic Mesothelioma

Cystic mesothelioma will be the rarest. It is found in peritoneal mesothelioma. More women will be affected by this than men.
This is incredibly rare and usually benign. It is found in women of childbearing age and is traditionally found in the abdomen. The disease forms a mesothelial-lined cyst that is surrounded by fibrous tissue. Surgery is the most common treatment.

Deciduoid Mesothelioma

This rare sub-type can occur in the abdomen of females who have not been exposed to asbestos, or in the lining of the lungs of men and women who have. This cancer is treated the same as regular epithelioid mesothelioma. But it can be extremely aggressive, depending on the shape of the cells.
Deciduoid mesothelioma will be a rare cell when it is found in pleural mesothelioma. It is actually more common when found in peritoneal, and it will be found in roughly 50% of cases. Deciduoid mesothelioma is a rare epithelial subtype that may be caused by factors other than asbestos exposure. This pattern features large round to polygonal cells with sharp borders.

Because it is so uncommon, deciduoid mesothelioma can be mistaken for other conditions. Including squamous cell carcinoma, anaplastic large cell lymphoma, gastrointestinal autonomic nerve tumor. Pseudotumoral deciduous, trophoblastic neoplasia and the oxyphilic variant of ovarian clear cell carcinoma.

Papillary Mesothelioma

Papillary mesothelioma is not going to be very aggressive but it is rare and may not be seen that often. It will be seen in the peritoneum. A slow-growing variant that is not prone to spread, well-differentiated papillary mesothelioma features papillae lined by a single layer of flat mesothelial cells. This type usually affects young women and is not related to asbestos exposure.

This very rare epithelioid mesothelioma occurs predominantly in the abdomens of women. It is technically cancerous, but it is not aggressive, and it rarely metastasizes. After surgery, most patients do not have a recurrence. It is sometimes called well-differentiated papillary mesothelioma (WDPM).
Small cell mesothelioma can be considered an additional subtype of mesothelioma, but it is typically diagnosed in biphasic tumors, which have a combination of both epithelial and sarcomatoid cells. In some cases,  multiple biopsies may be necessary to confirm a diagnosis. Physicians must carefully analyze cancer cells to understand the type of cancer that a patient has. In addition to being mistaken for other subtypes under the mesothelioma umbrella, these forms are commonly misdiagnosed as other diseases. A misdiagnosis can greatly hinder effective treatment and life expectancy.

Focus On What You Can Control

There are things you can and cannot control when it comes to your prognosis. You cannot control the cell type of your cancer or how it will ultimately impact your lifespan. But there are other prognostic factors you can control such as the treatments you choose and the lifestyle choices you make.
Participating in clinical trials, improving your diet and exercising regularly will boost your immune system. You can reach out to loved ones and join a support group. You can work with a palliative care specialist to manage symptoms and boost the quality of life.

These are a few of the steps you can take to help you live longer with epithelioid mesothelioma cancer. Along with proper treatment and the help of a mesothelioma specialist It is possible to outlive the average mesothelioma prognosis.

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Mesothelioma Master: Epithelioid Mesothelioma | Mesothelioma Master
Epithelioid Mesothelioma | Mesothelioma Master
Epithelioid mesothelioma is the most common type of mesothelioma. Those with epithelioid cells typically have better life expectancies and treatment.
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