Overview
Retiform hemangioendothelioma (RHE) is an extremely rare type of vascular tumor that falls into the category of borderline or low-grade malignant blood vessel tumors. The name "retiform" comes from the net-like pattern the tumor cells form when viewed under a microscope, resembling the rete testis (a network structure in the body). This tumor most commonly appears as a slow-growing skin lesion, often on the arms or legs, though it can occur in other locations. It typically presents as a raised, reddish or purplish plaque or nodule on the skin that may gradually enlarge over months to years. RHE is classified as a borderline vascular neoplasm, meaning it behaves somewhere between a benign (harmless) growth and a fully malignant (cancerous) tumor. While it tends to recur locally after surgical removal, it rarely spreads to distant parts of the body (metastasis). However, spread to nearby lymph nodes has been reported in some cases. Because of its rarity, there is no standardized treatment protocol. Surgery to completely remove the tumor with clear margins is the primary treatment approach. Radiation therapy has been used in some cases, particularly when complete surgical removal is not possible or when the tumor keeps coming back. The overall outlook is generally favorable compared to more aggressive vascular cancers, but long-term follow-up is important because of the tendency for local recurrence.
Key symptoms:
Slow-growing skin lump or raised patchReddish, purplish, or bluish skin discolorationPainless or mildly tender skin nodulePlaque-like skin growth on arms or legsSkin lesion that gradually increases in sizeSwelling in the affected areaRecurrence of the growth after removalEnlarged lymph nodes near the tumor in rare 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 Retiform hemangioendothelioma.
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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 Retiform 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.What stage is my tumor, and has it spread beyond the skin?,What type of surgery do you recommend, and what are the chances of complete removal?,How likely is it that the tumor will come back after surgery?,How often should I have follow-up exams and imaging after treatment?,Would radiation therapy be helpful in my case?,Are there any clinical trials or new treatments being studied for this type of tumor?,Should I seek a second opinion from a vascular tumor specialist?
Common questions about Retiform hemangioendothelioma
What is Retiform hemangioendothelioma?
Retiform hemangioendothelioma (RHE) is an extremely rare type of vascular tumor that falls into the category of borderline or low-grade malignant blood vessel tumors. The name "retiform" comes from the net-like pattern the tumor cells form when viewed under a microscope, resembling the rete testis (a network structure in the body). This tumor most commonly appears as a slow-growing skin lesion, often on the arms or legs, though it can occur in other locations. It typically presents as a raised, reddish or purplish plaque or nodule on the skin that may gradually enlarge over months to years. R
How is Retiform hemangioendothelioma inherited?
Retiform hemangioendothelioma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Retiform hemangioendothelioma?
15 specialists and care centers treating Retiform hemangioendothelioma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.