Overview
Composite hemangioendothelioma (CHE) is an extremely rare vascular tumor that falls into the category of borderline or intermediate-grade blood vessel tumors. This means it is not clearly benign (harmless) or clearly malignant (cancerous), but sits somewhere in between. The tumor is made up of a mixture of different types of vascular tissue patterns, which is why it is called "composite" — it combines features of several different vascular tumors within a single mass. These can include areas that look like retiform hemangioendothelioma, epithelioid hemangioendothelioma, angiosarcoma, or spindle cell hemangioma, all blended together. Composite hemangioendothelioma most commonly appears as a slow-growing lump or mass in the skin or soft tissues. It can occur in various parts of the body, including the arms, legs, head, and neck. The tumor may appear as a painless swelling, a discolored area of skin, or a firm nodule. In some cases, it can involve deeper tissues. While CHE generally has a low risk of spreading to distant parts of the body (metastasis), local recurrence after removal is relatively common, so careful follow-up is important. Treatment typically involves surgical removal of the tumor with clear margins. Because of the rarity of this condition, there is no standardized chemotherapy or radiation protocol, though these may be considered in cases where the tumor cannot be fully removed or if it recurs. Long-term monitoring by a specialist team is recommended to watch for any return of the tumor.
Also known as:
Key symptoms:
Slow-growing lump or mass under the skinPainless or mildly painful swellingSkin discoloration over the affected areaFirm nodule in the skin or soft tissueSwelling in the arms or legsLump on the head or neck areaRecurrent mass after previous removalEnlarged lymph nodes near the tumor in rare casesSkin ulceration over the mass in some cases
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 Composite hemangioendothelioma.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Composite hemangioendothelioma at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Composite hemangioendothelioma.
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 Composite hemangioendothelioma.
Community
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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.Was the tumor completely removed with clear margins?,What is the risk of the tumor coming back, and how will we monitor for recurrence?,Should I get a second opinion from a pathologist who specializes in vascular tumors?,Are there any additional treatments like radiation that I should consider?,How often should I have follow-up imaging, and what type of scans will be used?,Are there any clinical trials or research studies for this type of tumor?,What symptoms should I watch for that might indicate the tumor has returned or spread?
Common questions about Composite hemangioendothelioma
What is Composite hemangioendothelioma?
Composite hemangioendothelioma (CHE) is an extremely rare vascular tumor that falls into the category of borderline or intermediate-grade blood vessel tumors. This means it is not clearly benign (harmless) or clearly malignant (cancerous), but sits somewhere in between. The tumor is made up of a mixture of different types of vascular tissue patterns, which is why it is called "composite" — it combines features of several different vascular tumors within a single mass. These can include areas that look like retiform hemangioendothelioma, epithelioid hemangioendothelioma, angiosarcoma, or spindl
How is Composite hemangioendothelioma inherited?
Composite hemangioendothelioma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.