Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome

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ORPHA:313846OMIM:614564C10.9
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Overview

Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome is an extremely rare inherited condition that combines two main features: visible clusters of tiny, widened blood vessels on the skin (called telangiectasias) and a significantly increased risk of developing cancer in the oropharynx, which is the middle part of the throat including the base of the tongue, tonsils, and soft palate. The skin changes typically appear as small red or purple spots or fine lines on the skin surface, caused by dilated capillaries. These are usually painless but can be cosmetically concerning. The more serious aspect of this syndrome is the predisposition to oropharyngeal cancer. Affected family members may develop squamous cell carcinoma or other cancers in the throat area at higher rates than the general population. Symptoms of oropharyngeal cancer can include a persistent sore throat, difficulty swallowing, unexplained ear pain, a lump in the neck, or changes in voice. Because this syndrome is so rare, the treatment landscape is still evolving. Management focuses on regular cancer screening of the throat and oropharynx to catch any malignancy early, treatment of any cancers that develop (which may include surgery, radiation therapy, and chemotherapy), and monitoring and cosmetic management of skin telangiectasias. Genetic counseling is strongly recommended for affected families to understand their risks and plan appropriate surveillance.

Key symptoms:

Small red or purple spots on the skin from widened blood vesselsFine red lines or web-like patterns on the skinPersistent sore throatDifficulty swallowingUnexplained ear painLump or mass in the neckChanges in voice or hoarsenessUnexplained weight lossBleeding from the mouth or throatPain in the throat that does not go away

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome.

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No actively recruiting trials found for Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome at this time.

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No specialists are currently listed for Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome.

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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 Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome.

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Community

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Latest news about Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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.How often should I have screening exams for oropharyngeal cancer, and what do those exams involve?,Is genetic testing available for my family to identify who else may be at risk?,What specific symptoms should I watch for that might indicate early cancer development?,Are there any lifestyle changes I can make to reduce my cancer risk?,Should my children or other family members be screened, and at what age should screening begin?,What treatment options would be available if cancer is detected early?,Can you refer me to a genetic counselor who specializes in cancer predisposition syndromes?

Common questions about Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome

What is Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome?

Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome is an extremely rare inherited condition that combines two main features: visible clusters of tiny, widened blood vessels on the skin (called telangiectasias) and a significantly increased risk of developing cancer in the oropharynx, which is the middle part of the throat including the base of the tongue, tonsils, and soft palate. The skin changes typically appear as small red or purple spots or fine lines on the skin surface, caused by dilated capillaries. These are usually painless but can be cosmetically conc

How is Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome inherited?

Familial cutaneous telangiectasia and oropharyngeal cancer predisposition syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.