Epstein-Barr Virus-associated mesenchymal tumor

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Overview

Epstein-Barr Virus (EBV)-associated mesenchymal tumor is an extremely rare type of tumor that develops in the body's connective tissues (such as muscle, fat, or blood vessels) and is linked to infection with the Epstein-Barr virus. EBV is a very common virus — most people are infected at some point in their lives — but in rare cases, particularly in people with weakened immune systems, the virus can trigger the growth of unusual tumors in soft tissues. These tumors are sometimes also called EBV-positive smooth muscle tumors (EBV-SMTs) when they arise from smooth muscle cells. They can appear in various parts of the body, including the liver, lungs, spleen, gastrointestinal tract, and even the brain or spinal cord. Patients may have one tumor or multiple tumors at the same time. This condition is most commonly seen in people who are immunocompromised, such as organ transplant recipients on immunosuppressive medications, people living with HIV/AIDS, or individuals with congenital (inborn) immune deficiencies. Symptoms depend on where the tumor grows and may include pain, a noticeable lump, organ dysfunction, or general symptoms like fatigue and weight loss. Treatment typically involves surgery to remove the tumor when possible, along with efforts to improve the patient's immune function. Because this condition is so rare, there is no single standardized treatment protocol, and care is usually tailored to each individual patient. Reducing immunosuppressive therapy (when safe to do so) can sometimes help the body control the virus and slow tumor growth.

Also known as:

Key symptoms:

Painless or painful lump or mass in soft tissueAbdominal pain or swellingUnexplained weight lossFatigue and general weaknessLiver enlargement or liver massesLung nodules or breathing difficultiesBowel obstruction or digestive problemsNeurological symptoms if tumors are in the brain or spinal cordFever or night sweatsLoss of appetiteOrgan dysfunction depending on tumor location

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Epstein-Barr Virus-associated mesenchymal tumor.

View clinical trials →

No actively recruiting trials found for Epstein-Barr Virus-associated mesenchymal tumor at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Epstein-Barr Virus-associated mesenchymal tumor community →

Specialists

1 foundView all specialists →
HM
Hillard M. Lazarus, MD
CLEVELAND, OH
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Epstein-Barr Virus-associated mesenchymal tumor.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Epstein-Barr Virus-associated mesenchymal tumor

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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.Where exactly are the tumors located, and how many are there?,Is surgery an option for removing the tumor(s)?,Can my immunosuppressive medications be safely reduced to help fight the tumor?,How often will I need imaging scans to monitor the tumors?,Are there any clinical trials or experimental treatments available for this condition?,What symptoms should prompt me to seek emergency care?,How will this diagnosis affect my long-term health and quality of life?

Common questions about Epstein-Barr Virus-associated mesenchymal tumor

What is Epstein-Barr Virus-associated mesenchymal tumor?

Epstein-Barr Virus (EBV)-associated mesenchymal tumor is an extremely rare type of tumor that develops in the body's connective tissues (such as muscle, fat, or blood vessels) and is linked to infection with the Epstein-Barr virus. EBV is a very common virus — most people are infected at some point in their lives — but in rare cases, particularly in people with weakened immune systems, the virus can trigger the growth of unusual tumors in soft tissues. These tumors are sometimes also called EBV-positive smooth muscle tumors (EBV-SMTs) when they arise from smooth muscle cells. They can appear i

How is Epstein-Barr Virus-associated mesenchymal tumor inherited?

Epstein-Barr Virus-associated mesenchymal tumor follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Epstein-Barr Virus-associated mesenchymal tumor?

1 specialists and care centers treating Epstein-Barr Virus-associated mesenchymal tumor are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.