Description

Epithelioid Sarcoma is a rare type of cancer that begins as a growth of cells in soft tissues. It can happen in any part of the body. Often begins beneath the skin on the finger, hand, forearm, knee, or bottom of the leg.

Epithelioid Sarcoma can occur at any age. It most often affects adolescents and young to middle-aged adults. Epithelioid Sarcoma tends to grow slowly. You can come back after treatment.

Epithelioid Sarcoma is a type of cancer called soft tissue sarcoma. These cancers occur in the body of the connective tissues. There are many types of soft tissue sarcoma. Sarcomas of soft tissues, including the epithelioid sarcoma, are not common. It is best to seek care at a cancer center that has experience in the treatment of people with sarcoma.

Symptoms

Epithelioid Sarcoma symptoms include a small growing company under the skin called a nodule. The lump is usually not painful or tender. Sometimes there is more than one nodule. Sometimes the nodule form an ulcer on the skin that do not heal.

The nodules caused by epithelioid sarcoma most often grow in the fingers, hands or forearms. It can also grow in the knee or the lower leg.

Causes

The cause of epithelioid sarcoma is not known. This type of cancer causes a growth of cells in soft tissues.

Epithelioid Sarcoma starts when cells of the soft tissues to achieve changes in their DNA. A cell's DNA contains the instructions that tell the cell what to do. In healthy cells, the DNA contains the instructions to grow and multiply at a set rate. The instructions that tell cells to die at a set time. In cancer cells, changes in DNA to give different instructions. The changes tell cancer cells to grow and multiply quickly. Cancer cells can continue living when healthy cells would die. This makes too many cells.

The cancer cells can form a tumor that can be felt through the skin. Cells can break away and spread to the lymph nodes and to other parts of the body. When cancer spreads, it is called metastatic cancer.

Risk factors

The risk of epithelioid sarcoma is greater in younger people. This type of cancer can occur at any age. But it occurs more often in adolescents and young to middle-aged adults. It is less common in children and older adults.

There is no way to prevent the epithelioid sarcoma.

Diagnosis

Epithelioid Sarcoma can be difficult to diagnose. It seems that the problems are much more common. Often health care professionals to consider the most common problems in the first place. For example, a skin ulcer that is not healing could be mistaken for an infection of the skin.

Tests and procedures used in the diagnosis of epithelioid sarcoma include:

  • Imaging tests. Imaging tests make pictures of the body. You can show the location and size of an epithelioid sarcoma. The tests may include X-rays, magnetic resonance imaging, computed tomography and positron emission tomography, also called a PET scan.
  • To obtain tissue for analysis. A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue can be removed through a needle placed through the skin and the cancer. Sometimes surgery is necessary to obtain a tissue sample. The sample is analyzed in a laboratory to see if it is cancer. Other special tests to give more details on the cancer cells. Your health care team uses this information to make a treatment plan.

Treatment

Surgery is the most common treatment for epithelioid sarcoma. Sometimes, other treatments may be used in addition to surgery. Treatment options may include:

  • Surgery. The surgery involves removing the cancer and some healthy tissue around it. Taking some healthy tissue helps to ensure that all cancerous cells are removed. Get all of the cancer cells, reduces the risk that the cancer will come back.
  • The radiation therapy. Radiation therapy uses high-energy rays to kill cancer cells. Radiation therapy is sometimes used before surgery to reduce the size of the tumor. This may make it more likely that all of the cancer will be removed during surgery. Radiation therapy may be used after surgery to destroy any cancer cells that may be left.
  • The targeted therapy. Targeted therapy uses drugs that attack specific chemicals in cancer cells that help them grow. By blocking these chemicals, specific treatments can cause cancer cells to die. Targeted therapy may be an option if you can't have the surgery, or if other treatments do not work. A targeted therapy used for epithelioid sarcoma is tazemetostat (Tazverik).
  • Chemotherapy. Chemotherapy uses strong drugs to kill cancer cells. Chemotherapy may be an option for the treatment of epithelioid sarcoma that has spread to other parts of the body. It can also be used when surgery is not an option.
  • Clinical trials. Clinical trials are studies of new treatments. These studies provide an opportunity to try the latest treatment options. Side effects may not be known. Ask your health care team if you can be able to take part in a clinical trial.

Preparing for your appointment

Make an appointment with a doctor or other health care professional if you have any symptoms that worry you. If your doctor thinks you may have epithelioid sarcoma, that person can refer you to a specialist. Often, this is a doctor who specializes in cancer, called an oncologist.

Appointments can be brief, and being prepared can help. Here is some information that can help you prepare for your visit.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test. Make a list of:

  • Your symptoms, including any that seem unrelated to the reason for the appointment.
  • Key personal information, including recent changes in the life and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care professional.

For epithelioid sarcoma, some basic questions to ask your health care professional include:

  • Do I have cancer?
  • I need more tests?
  • What are my treatment options?
  • What are the potential risks of these treatment options?
  • Do any of the treatments to cure cancer?
  • I have a copy of the pathology report?
  • How much time can I take to consider the treatment options?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?
  • What would happen if I decide not to have treatment?

Do not hesitate to ask other questions.

What to expect from your doctor

Your healthcare provider is likely to ask several questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How bad are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Epithelioid sarcoma