Overview
Extraskeletal Ewing sarcoma (also called extraosseous Ewing sarcoma or soft tissue Ewing sarcoma) is a rare and aggressive type of cancer that belongs to the Ewing sarcoma family of tumors. Unlike the more common form of Ewing sarcoma that starts in bones, extraskeletal Ewing sarcoma develops in the soft tissues of the body, such as muscles, fat, or connective tissue. It can appear in many locations, including the chest wall, abdomen, pelvis, arms, legs, and head and neck region. This cancer most often affects children, teenagers, and young adults, though it can occur at any age. Common symptoms include a growing lump or mass that may or may not be painful, swelling in the affected area, and general symptoms like fever, fatigue, and weight loss. Because the tumor can grow in many different body locations, symptoms vary depending on where the tumor develops. For example, a tumor in the chest may cause difficulty breathing, while one in the abdomen may cause stomach pain. Treatment typically involves a combination of chemotherapy, surgery, and sometimes radiation therapy. Chemotherapy is usually given first to shrink the tumor before surgery, and additional chemotherapy follows to kill any remaining cancer cells. The treatment approach is generally similar to that used for bone-based Ewing sarcoma. Advances in treatment have improved survival rates significantly over the past few decades, but outcomes depend on factors such as tumor size, location, and whether the cancer has spread at the time of diagnosis.
Key symptoms:
A growing lump or mass in soft tissuePain or tenderness at the tumor siteSwelling in the affected areaUnexplained feverFatigue and tirednessUnintended weight lossDifficulty breathing if the tumor is in the chestAbdominal pain if the tumor is in the belly areaNumbness or tingling if the tumor presses on nervesDifficulty moving a limb near the tumorNight sweatsLoss of appetite
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Extraskeletal Ewing sarcoma.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Extraskeletal Ewing sarcoma at this time.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Extraskeletal Ewing sarcoma.
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1 articlesCaregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the exact location and size of the tumor, and has it spread to other parts of the body?,What treatment plan do you recommend, and what are the expected side effects of each treatment?,Should we seek a second opinion at a sarcoma specialty center?,Are there any clinical trials available that might be appropriate for this case?,What are the long-term side effects of treatment, including effects on fertility and heart health?,How will we monitor for recurrence after treatment is completed?,What support services are available for emotional, nutritional, and rehabilitation needs during and after treatment?
Common questions about Extraskeletal Ewing sarcoma
What is Extraskeletal Ewing sarcoma?
Extraskeletal Ewing sarcoma (also called extraosseous Ewing sarcoma or soft tissue Ewing sarcoma) is a rare and aggressive type of cancer that belongs to the Ewing sarcoma family of tumors. Unlike the more common form of Ewing sarcoma that starts in bones, extraskeletal Ewing sarcoma develops in the soft tissues of the body, such as muscles, fat, or connective tissue. It can appear in many locations, including the chest wall, abdomen, pelvis, arms, legs, and head and neck region. This cancer most often affects children, teenagers, and young adults, though it can occur at any age. Common sympt
How is Extraskeletal Ewing sarcoma inherited?
Extraskeletal Ewing sarcoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Extraskeletal Ewing sarcoma?
25 specialists and care centers treating Extraskeletal Ewing sarcoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.