What is the definition of Epithelioid Sarcoma?

Epithelioid sarcoma (ES) is a rare cancerous tumor that most often occurs in the soft tissue of the fingers, hands and forearms of young adults. It can also occur elsewhere in the body. ES usually begins as a painless, firm growth or bump that may be accompanied by an open wound (ulceration) in the skin covering the growth. This type of tumor often comes back after treatment or spreads to other parts the body (metastasis). The cause of epithelioid sarcoma is unknown. It is diagnosed by a clinical examination and by testing a small sample of the tumor (biopsy) in a laboratory. Epithelioid sarcoma is treated with surgery to remove all the cancer cells (wide local excision) and sometimes with radiation therapy. Chemotherapy may also be used if the cancer has spread to other parts of the body.

What are the causes for Epithelioid Sarcoma?

The exact cause of epithelioid sarcoma is unknown. About 30% of people have had a previous trauma at the site where the tumor grows, but it is unclear if this has any significance.

What are the symptoms for Epithelioid Sarcoma?

The following list includes the most common signs and symptoms in people with epithelioid sarcoma (ES). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list also does not include every symptom or feature that has been described in this condition.

Some of the symptoms may include:
  • Painless growth on the extremities
  • Break on the skin (ulceration)
Often people with epithelioid sarcoma have few or no other symptoms. ES typically occurs in young adults, but it can occur at any age.  Males are more often affected than females. ES is an aggressive tumor and often comes back after treatment or spreads to other parts of the body. The long term outcome depends on how aggressive the tumor is.

What are the current treatments for Epithelioid Sarcoma?

Epithelioid sarcoma (ES) is usually treated with surgery to remove the tumor and surrounding tissue. In some cases, radiation and chemotherapy may be used.  ES is an aggressive tumor and often grows back after surgical removal. It also has a high risk of spreading to other parts of the body (metastasis).

Specialists who may be involved in the care of someone with ES include:
  • Oncologist
  • Radiation oncologist
  • Surgeon
  • Pathologist

How is Epithelioid Sarcoma diagnosed?

Epithelioid sarcoma is diagnosed based on a clinical examination and an examination of a piece of the tumor in the laboratory (biopsy).
  • Journal: Journal of medical case reports
  • Published —
Primary pulmonary epithelioid sarcoma: a case report.
  • Condition: Treatment-Naive Programmed Death-Ligand 1 Positive Metastatic Soft Tissue Sarcomas (MSTS)
  • Journal: BMC cancer
  • Treatment Used: Nivolumab plus Ipilimumab (NPI) vs. Nivolumab Alone
  • Number of Patients: 0
  • Published —
This study assessed the safety and effectiveness of nivolumab plus ipilimumab (NPI) versus nivolumab alone (NIV) in patients with treatment-naive programmed death-ligand 1 (PD-L1) positive metastatic soft tissue sarcomas (MSTS).
Clinical Trial
  • Status: Not yet recruiting
  • Phase: Phase 1
  • Intervention Type: Drug
  • Participants: 32
  • Start Date: December 2021
B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)
Clinical Trial
  • Status: Not yet recruiting
  • Phase: Early Phase 1
  • Intervention Type: Drug
  • Participants: 20
  • Start Date: November 1, 2021
A Pilot Study of Gemcitabine Plus High-Dose Ascorbate in Locally Advanced Unresectable or Metastatic Soft Tissue and Bone Sarcomas Including Adolescents