Overview
Rare odontogenic tumors are a group of uncommon growths that develop from the tissues involved in tooth formation. These tumors arise in the jawbones or the soft tissues surrounding the teeth. The term "odontogenic" means they come from cells that normally help create teeth, including the enamel, dentin, or other dental structures. Because there are many different types of cells involved in making teeth, there are several subtypes of rare odontogenic tumors, each with its own behavior and characteristics. These tumors can cause swelling of the jaw, pain, loose teeth, difficulty chewing, or may be found by chance on a dental X-ray when no symptoms are present. Some rare odontogenic tumors grow slowly and are benign (not cancerous), while others can be locally aggressive, destroying nearby bone and tissue. In very rare cases, some types may become malignant (cancerous). Treatment usually involves surgery to remove the tumor. The extent of surgery depends on the tumor type, size, and location. Some tumors can be treated with a simple removal (enucleation or curettage), while more aggressive types may require removal of a section of the jawbone followed by reconstruction. Regular follow-up with imaging is important because some types have a tendency to come back. Radiation therapy or chemotherapy is rarely needed and is generally reserved for the uncommon malignant forms. Early detection through routine dental care and imaging can lead to better outcomes.
Key symptoms:
Swelling of the jaw or facePain in the jaw or teethLoose teeth without an obvious causeA lump or mass in the gums or jawboneDifficulty opening the mouthDifficulty chewing or bitingNumbness or tingling in the lip or chinTeeth that do not come in as expectedShifting or displacement of teethA growth found on a dental X-ray with no symptomsRecurrent infections in the jaw areaChanges in how the teeth fit together when biting
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
3 eventsShanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University — PHASE2, PHASE3
Alexandria University — NA
Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Rare odontogenic tumor.
2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
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 Rare odontogenic 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.What specific type of odontogenic tumor do I have, and is it benign or malignant?,What surgical approach do you recommend, and what are the risks?,What is the chance that this tumor will come back after treatment?,How often will I need follow-up imaging, and for how long?,Will I need jaw reconstruction or dental rehabilitation after surgery?,Are there any clinical trials or new treatments available for my type of tumor?,Should I be tested for any genetic syndromes that are associated with this type of tumor?
Common questions about Rare odontogenic tumor
What is Rare odontogenic tumor?
Rare odontogenic tumors are a group of uncommon growths that develop from the tissues involved in tooth formation. These tumors arise in the jawbones or the soft tissues surrounding the teeth. The term "odontogenic" means they come from cells that normally help create teeth, including the enamel, dentin, or other dental structures. Because there are many different types of cells involved in making teeth, there are several subtypes of rare odontogenic tumors, each with its own behavior and characteristics. These tumors can cause swelling of the jaw, pain, loose teeth, difficulty chewing, or ma
How is Rare odontogenic tumor inherited?
Rare odontogenic tumor follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Rare odontogenic tumor?
Yes — 2 recruiting clinical trials are currently listed for Rare odontogenic tumor on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Rare odontogenic tumor?
1 specialists and care centers treating Rare odontogenic tumor are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.