Overview
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that most often grows slowly under the skin or in deeper soft tissues. It is sometimes called angiomatoid malignant fibrous histiocytoma, though this older name is no longer preferred. AFH is considered a tumor of uncertain or low malignant potential, meaning it sits somewhere between a fully benign (harmless) growth and a fully malignant (cancerous) one. It most commonly appears in children, teenagers, and young adults, though it can occur at any age. The tumor usually shows up as a painless lump, most often on the arms, legs, or trunk. It tends to grow slowly and is often surrounded by a fibrous shell and blood-filled spaces, which gives it its name. Some people also experience whole-body symptoms like fever, weight loss, and anemia, which can make it look like other illnesses at first. Treatment usually involves surgery to remove the tumor completely. When the tumor is fully removed, most people do well and the outlook is generally favorable. However, AFH can come back locally or, rarely, spread to other parts of the body, so follow-up care is important. Research into targeted therapies is ongoing, especially for cases that cannot be fully removed with surgery.
Also known as:
Key symptoms:
Painless lump or swelling under the skinSlow-growing mass on the arms, legs, or trunkUnexplained feverUnintended weight lossFatigue or low energyAnemia (low red blood cell count)Swollen lymph nodes nearbyOccasional mild pain or tenderness at the lump siteRarely, a lump in deeper tissues that is harder to feel
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
1 eventSt. Jude Children's Research Hospital — PHASE1, PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Angiomatoid fibrous histiocytoma.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →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 Angiomatoid fibrous histiocytoma.
Community
No community posts yet. Be the first to share your experience with Angiomatoid fibrous histiocytoma.
Start the conversation →Latest news about Angiomatoid fibrous histiocytoma
Disease timeline:
New recruiting trial: NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
A new clinical trial is recruiting patients for Angiomatoid fibrous histiocytoma
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.Was the tumor completely removed, and what do the surgical margins show?,What specific gene fusion was found in my tumor, and what does that mean for my treatment and follow-up?,How often do I need follow-up imaging, and for how long?,What signs of recurrence should I watch for at home?,Are there any clinical trials I should know about in case the tumor comes back?,Should I see a specialist at a sarcoma center for a second opinion?,Is there any risk that other family members could develop this tumor?
Common questions about Angiomatoid fibrous histiocytoma
What is Angiomatoid fibrous histiocytoma?
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that most often grows slowly under the skin or in deeper soft tissues. It is sometimes called angiomatoid malignant fibrous histiocytoma, though this older name is no longer preferred. AFH is considered a tumor of uncertain or low malignant potential, meaning it sits somewhere between a fully benign (harmless) growth and a fully malignant (cancerous) one. It most commonly appears in children, teenagers, and young adults, though it can occur at any age. The tumor usually shows up as a painless lump, most often on the arms, legs
How is Angiomatoid fibrous histiocytoma inherited?
Angiomatoid fibrous histiocytoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Angiomatoid fibrous histiocytoma?
Yes — 1 recruiting clinical trial is currently listed for Angiomatoid fibrous histiocytoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Angiomatoid fibrous histiocytoma?
17 specialists and care centers treating Angiomatoid fibrous histiocytoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.