Epithelioid Sarcoma

Origin and Causes

Epithelioid sarcoma is a rare soft tissue cancer that accounts for 1% of all sarcomas. Adolescents and young adults are most at risk of developing epithelioid sarcoma. To date, no cause has been identified to explain the occurrence of this cancer. The two subtypes of epithelioid sarcoma are proximal type and distal type.

Symptoms and Signs

Epithelioid sarcoma can occur in any part of the body, but is mostly found in the upper extremities. It most commonly presents as a slow-growing, painless mass. Epithelioid sarcoma is an aggressive cancer that can cause metastases, mainly in the lungs and lymph nodes.

Diagnosis

When a sarcoma is suspected, the initial evaluation includes either a CT scan or MRI. The diagnosis is made by biopsy of the suspected area. The investigation may be completed by a chest CT scan and a PET scan.

Treatment

When an epithelioid sarcoma is localized, the treatment of choice is surgery to remove all of it. Radiation therapy before or after surgery is often necessary.

In cases where metastases are found, chemotherapy is offered with the goal of controlling symptoms and disease progression. The preferred agents are doxorubicin-ifosfamide, gemcitabine-docetaxel, and vinorelbine. Clinical trials may also be explored.

Follow-up

After surgery, patients with epithelioid sarcoma will be closely monitored due to the risk of recurrence (when the cancer returns).