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.