Print OverviewEpithelioid 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. Products & ServicesA Book: Mayo Clinic Family Health BookNewsletter: Mayo Clinic Health Letter — Digital EditionShow more products from Mayo Clinic SymptomsEpithelioid 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. Request an appointment CausesThe 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 factorsThe 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. By Mayo Clinic Staff Request an appointment Diagnosis & treatment Jan. 17, 2025 Print Living with epithelioid sarcoma? Connect with others like you for support and answers to your questions in the Sarcoma support group on Mayo Clinic Connect, a patient community. Sarcoma Discussions Does anyone have chondrosarcoma? 23 Replies Sat, Jan 18, 2025 chevron-right How do you, as a parent, deal with the anger? Son has sarcoma 9 Replies Sat, Jan 18, 2025 chevron-right GNET Sarcoma-Gastrointestinal Neuroectodermal Tumors 15 Replies Thu, Jan 16, 2025 chevron-right See more discussions Show references Goldblum JR, et al. Other malignant soft tissue tumors, including those of uncertain type. In: Enzinger and Weiss's Soft Tissue Tumors. 7th ed. Elsevier; 2020. https://www.clinicalkey.com. Accessed Jan. 5, 2023. Schuetze SM, et al. Uncommon sarcoma subtypes. https://www.uptodate.com/contents/search. Accessed Jan. 5, 2023. Sarcomas, soft tissue — Introduction. Cancer.Net. https://www.cancer.net/cancer-types/sarcoma/view-all. Accessed Jan. 5, 2023. Soft tissue sarcoma. National Comprehensive Cancer Network. https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1464. Accessed Dec. 20, 2024. Meissner M, et al. Pharmacotherapeutic strategies for epithelioid sarcoma: Are we any closer to a non-surgical cure? Expert Opinion on Pharmacotherapy. 2023; doi:10.1080/14656566.2023.2224500. Gounder M, et al. Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: An international, open-label, phase 2 basket study. The Lancet Oncology. 2020; doi:10.1016/s1470-2045(20)30451-4. Related Products & Services A Book: Mayo Clinic Family Health Book Newsletter: Mayo Clinic Health Letter — Digital Edition Show more products and services from Mayo Clinic Epithelioid sarcomaSymptoms&causesDiagnosis&treatmentDoctors&departments Advertisement Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission. Advertising & Sponsorship Policy Opportunities Ad Choices Mayo Clinic Press Check out these best-sellers and special offers on books and newsletters from Mayo Clinic Press. 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