Epithelioid sarcoma
Overview
Epithelioid sarcoma is a rare type of cancer that starts as a growth of cells in the soft tissue. It can happen anywhere on the body. It often starts under the skin on the finger, hand, forearm, knee or lower leg.
Epithelioid sarcoma can happen at any age. It most often affects teenagers and young to middle-aged adults. Epithelioid sarcoma tends to grow slowly. It can come back after treatment.
Epithelioid sarcoma is a type of cancer called a soft tissue sarcoma. These cancers happen in the body's connective tissues. There are many types of soft tissue sarcoma. Soft tissue sarcomas, including epithelioid sarcoma, are not common. It is best to seek care at a cancer center that has experience treating people with sarcoma.
Symptoms
Epithelioid sarcoma symptoms include a small, firm growth under the skin called a nodule. The nodule usually isn't painful or tender. Sometimes there is more than one nodule. Sometimes the nodule forms a sore on the skin that won't heal.
The nodules caused by epithelioid sarcoma most often grow on the fingers, hands or forearms. They also may grow on the knee or lower leg.
Causes
The cause of epithelioid sarcoma isn't known. This cancer causes a growth of cells in the soft tissue.
Epithelioid sarcoma starts when soft tissue cells get changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.
The cancer cells might form a growth that can be felt through the skin. The cells can break away and spread to lymph nodes and to other parts of the body. When cancer spreads, it's called metastatic cancer.
Risk factors
The risk of epithelioid sarcoma is higher in younger people. This cancer can happen at any age. But it happens most often in adolescents and young to middle-aged adults. It is less common in children and older adults.
There is no way to prevent epithelioid sarcoma.
Diagnosis
Epithelioid sarcoma can be hard to diagnose. It looks like problems that are much more common. Often healthcare professionals consider those more common problems first. For example, a sore on the skin that is not healing could be mistaken for a skin infection.
Tests and procedures used in the diagnosis of epithelioid sarcoma include:
Treatment
Surgery is the most common treatment for epithelioid sarcoma. Sometimes other treatments may be used in addition to surgery. Treatment options may include:
Preparing for an appointment
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your health professional thinks you may have epithelioid sarcoma, that person may refer you to a specialist. Often, this is a doctor who specializes in cancer, called an oncologist.
Appointments can be short and being prepared can help. Here's some information that may help you get ready for your visit.
When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:
For epithelioid sarcoma, some basic questions to ask your healthcare professional include:
Don't hesitate to ask other questions.
Your healthcare professional is likely to ask you several questions, such as:
Updated on Jan 17, 2025
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