Introduction
Epithelioid sarcoma is a rare cancer that begins as uncontrolled cellular growth in the soft tissue. It can occur in any part of the body. However, it often starts under the skin of the hand, finger, arm, knee, or leg. Epithelioid sarcoma leads to the formation of a small lump under the skin called a nodule which is often asymptomatic. There can be one or several nodules. The growth often causes sores on the skin that do not heal.
Epithelioid sarcoma mostly affects young adults and teenagers. However, it can also affect older adults. Epithelioid sarcoma often grows slowly and recurs after treatment. Epithelioid sarcoma is also known as soft tissue sarcoma. These occur in the connective tissues of the body. Soft tissue sarcomas are of many types and are usually uncommon; thus, the treatment should be at a hospital experienced in treating people with sarcoma. The outlook is positive if treated on time.
What Causes Epithelioid Sarcoma?
The exact cause of what causes epithelioid sarcoma is unknown. However, it is believed to be related to an abnormality of the SMARCB1 gene, which instructs the body on how to make protein. This gene alteration or mutation is acquired; it occurs with time and is not something one is born with. Like other soft tissue sarcomas, epithelioid sarcoma often develops without any particular reason. People who received radiation therapy treatment for other cancers have a slightly increased risk for epithelioid sarcoma. In addition, people with a family history of certain types of cancer have a slightly higher risk of developing epithelioid sarcoma.
Such cancers include the following:
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Neurofibromatosis - A genetic disorder of the nervous system.
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Retinoblastoma - Eye cancer.
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Li-Fraumeni Syndrome - A genetic disorder that increases the risk of developing cancer.
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Werner Syndrome - A genetic disorder causing rapid aging and an increased risk of developing cancer.
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Gardner Syndrome - An autosomal dominant disease with multiple polyps in the gastrointestinal tract.
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Tuberous Sclerosis Complex - A rare genetic disease that leads to the growth of tumors in various body parts.
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Gorlin Syndrome - A rare genetic disease affecting many body organs and tissues.
What Are the Different Types of Epithelioid Sarcoma?
Epithelioid sarcoma is of the following two types:
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Distal-Type - The distal type of epithelioid sarcoma is the most common type. It usually affects body parts such as the hands, feet, and legs.
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Proximal-Type - The proximal type of epithelioid sarcoma is the uncommon form of epithelioid sarcoma. It usually affects the chest, abdomen, armpits, genitals, head, and neck. Compared to the distal type, the proximal-type epithelioid sarcoma is more aggressive and more challenging to treat.
What Are the Symptoms of Epithelioid Sarcoma?
Epithelioid sarcoma is usually asymptomatic in the earlier stages. However, as the tumor grows, one may begin to notice the following symptoms:
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A small to large new lump or growth.
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Ulcerations over the lump.
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Painful lump.
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A lump that increases in size.
How Is Epithelioid Sarcoma Diagnosed?
Epithelioid sarcoma is difficult to diagnose since it resembles other common medical problems. For example, a non-healing skin sore could be mistaken for a skin infection.
The following tests and procedures are used to diagnose epithelioid sarcoma:
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Imaging Tests: These tests include X-ray, MRI (magnetic resonance imaging), CT (computed tomography), and PET (positron emission tomography) scans. They can detect the location and size of epithelioid sarcoma.
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Biopsy: A biopsy is a procedure to remove some of the diseased tissue for testing in a laboratory. The tissue might be taken with the help of a needle into the tumor. Sometimes surgery is required to get the tissue sample. Then, the sample is tested in a laboratory to see if it is cancerous. Various other tests give more details about cancer cells. The doctor uses this information to chalk a treatment plan.
How Is Epithelioid Sarcoma Treated?
The most common treatment for epithelioid sarcoma is surgery. However, other treatments may be used in addition to surgery sometimes.
The following are the treatment options:
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Surgery - Surgery involves removing the cancerous growth and some of the healthy tissues around it. Removing a part of the healthy tissues and the tumor ensures that all the cancer cells are removed. This lowers the risk of cancer recurrence.
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Radiation Therapy - Radiation therapy uses powerful radiation to kill cancer cells. Radiation therapy is usually used before surgery to reduce the tumor size. This is done so that the cancerous growth can be removed during surgery. Radiation therapy may be used post-surgery to kill any cancer cells that might be left.
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Targeted Therapy -Targeted therapy is a therapy that uses medicines that attack particular compounds in the cancer cells that help them grow. Targeted therapy blocks these chemicals or compounds, causing the cancer cells to die. Targeted therapy is an option if one cannot undergo surgery or other treatment modalities do not work.
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Chemotherapy - Chemotherapy is the use of strong medications to kill cancer cells. Chemotherapy treats epithelioid sarcoma that has spread to other body parts. It can also be used when surgery is not an option for treatment.
What Is the Prognosis of Epithelioid Sarcoma?
The stage of the disease usually determines the prognosis of epithelioid sarcoma. It is associated with invasion of the blood vessels, tumor size, resectability, and metastases or spread to other body parts. Large tumor size and early spread are associated with poor outcomes.
The proximal type of epithelioid sarcoma is the aggressive form of the tumor. Gender, age, area, tumor size, and microscopic findings affect the prognosis of the tumor. Female patients are found to have better outcomes. The distal epithelioid sarcoma has been found to have better outcomes than the proximal ones. Detection at an earlier age has a better prognosis. Tumors over two centimeters in diameter, necrosis, and vascular invasion have been associated with poor outcomes.
Conclusion
Epithelioid sarcoma is a slow-growing mass of tissue usually found in the extremities. Since epithelioid sarcoma is benign, an efficient physician must detect it. Then, an appropriate treatment plan and long-term disease management are chalked out. A positive outcome can be achieved after complete resection. Radiation therapy is often used. Palliative treatment is often used.
