Overview
Pleomorphic adenoma of the salivary gland, also known as benign mixed tumor, is the most common type of tumor that develops in the salivary glands. The term 'OBSOLETE' in the Orphanet classification indicates that this entry has been retired or reclassified, likely because pleomorphic adenoma is not considered a rare disease in the traditional sense — it is actually the most frequent salivary gland tumor, accounting for roughly 60-70% of all salivary gland tumors. These tumors most often arise in the parotid gland (the large salivary gland located in front of the ear), but they can also occur in the submandibular gland or in minor salivary glands throughout the mouth and throat. Pleomorphic adenomas are benign (non-cancerous) tumors, but they can grow slowly over time and, if left untreated for many years, carry a small risk of transforming into a malignant (cancerous) tumor called carcinoma ex pleomorphic adenoma. The tumor typically presents as a painless, slow-growing lump or swelling near the jaw, cheek, or inside the mouth. Treatment is primarily surgical, and the prognosis after complete removal is generally excellent. However, these tumors can recur if not fully removed, which is why careful surgical technique is important. Because this condition is relatively common and not classified as a rare disease, its Orphanet entry has been marked as obsolete.
Key symptoms:
Painless lump or swelling near the ear or jawSlow-growing mass in the cheek areaSwelling inside the mouth or on the palateFirm, movable lump under the skinFacial asymmetry due to tumor growthRarely, facial nerve weakness if the tumor is largeDifficulty swallowing if the tumor is in the throat areaDiscomfort or fullness in the affected area
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
1 eventAvacta Life Sciences Ltd — PHASE1
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for OBSOLETE: Pleomorphic salivary gland adenoma.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Specialists
View all specialists →No specialists are currently listed for OBSOLETE: Pleomorphic salivary gland adenoma.
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 OBSOLETE: Pleomorphic salivary gland adenoma.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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 type of surgery do you recommend, and what are the risks specific to my tumor location?,What is the risk of facial nerve damage during surgery, and how would that be managed?,How likely is it that this tumor could come back after surgery?,How often will I need follow-up appointments and imaging after surgery?,Is there any chance this tumor could become cancerous if I delay treatment?,What side effects should I expect after surgery, and how long is the recovery?,Should I get a second opinion from a head and neck surgery specialist?
Common questions about OBSOLETE: Pleomorphic salivary gland adenoma
What is OBSOLETE: Pleomorphic salivary gland adenoma?
Pleomorphic adenoma of the salivary gland, also known as benign mixed tumor, is the most common type of tumor that develops in the salivary glands. The term 'OBSOLETE' in the Orphanet classification indicates that this entry has been retired or reclassified, likely because pleomorphic adenoma is not considered a rare disease in the traditional sense — it is actually the most frequent salivary gland tumor, accounting for roughly 60-70% of all salivary gland tumors. These tumors most often arise in the parotid gland (the large salivary gland located in front of the ear), but they can also occur
How is OBSOLETE: Pleomorphic salivary gland adenoma inherited?
OBSOLETE: Pleomorphic salivary gland adenoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does OBSOLETE: Pleomorphic salivary gland adenoma typically begin?
Typical onset of OBSOLETE: Pleomorphic salivary gland adenoma is adult. Age of onset can vary across affected individuals.
Are there clinical trials for OBSOLETE: Pleomorphic salivary gland adenoma?
Yes — 1 recruiting clinical trial is currently listed for OBSOLETE: Pleomorphic salivary gland adenoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.