Benign epithelial tumor of salivary glands

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ORPHA:276148OMIM:181030D11.0D11.7
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Overview

Benign epithelial tumors of the salivary glands are a group of non-cancerous growths that arise from the epithelial (lining) cells of the major or minor salivary glands. The major salivary glands include the parotid glands (located in front of the ears), the submandibular glands (beneath the jaw), and the sublingual glands (under the tongue), while minor salivary glands are distributed throughout the oral cavity and upper aerodigestive tract. The most common type is pleomorphic adenoma (also called benign mixed tumor), which accounts for the majority of salivary gland neoplasms. Other subtypes include Warthin tumor (papillary cystadenoma lymphomatosum), basal cell adenoma, canalicular adenoma, oncocytoma, and myoepithelioma. These tumors typically present as slow-growing, painless swellings in the affected salivary gland region, most frequently in the parotid gland. They generally affect one side and are firm, mobile, and well-circumscribed on examination. While benign, some subtypes — particularly pleomorphic adenoma — carry a small risk of malignant transformation (carcinoma ex pleomorphic adenoma) if left untreated over many years. Symptoms may include facial asymmetry, a palpable mass in the cheek or neck, and occasionally discomfort or difficulty swallowing if the tumor grows large enough to compress surrounding structures. Facial nerve involvement causing weakness is uncommon in benign tumors and should raise concern for malignancy. Diagnosis typically involves imaging studies such as ultrasound, CT, or MRI, along with fine-needle aspiration cytology to characterize the tumor. The primary treatment is surgical excision, with the specific approach depending on the tumor's location. For parotid tumors, superficial or total parotidectomy with facial nerve preservation is the standard of care. For submandibular gland tumors, gland excision is performed. Prognosis is generally excellent, though pleomorphic adenomas have a known recurrence risk, particularly if incompletely excised or if tumor spillage occurs during surgery. Long-term follow-up is recommended to monitor for recurrence or rare malignant transformation.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Benign epithelial tumor of salivary glands.

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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

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Common questions about Benign epithelial tumor of salivary glands

What is Benign epithelial tumor of salivary glands?

Benign epithelial tumors of the salivary glands are a group of non-cancerous growths that arise from the epithelial (lining) cells of the major or minor salivary glands. The major salivary glands include the parotid glands (located in front of the ears), the submandibular glands (beneath the jaw), and the sublingual glands (under the tongue), while minor salivary glands are distributed throughout the oral cavity and upper aerodigestive tract. The most common type is pleomorphic adenoma (also called benign mixed tumor), which accounts for the majority of salivary gland neoplasms. Other subtypes

How is Benign epithelial tumor of salivary glands inherited?

Benign epithelial tumor of salivary glands follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Benign epithelial tumor of salivary glands typically begin?

Typical onset of Benign epithelial tumor of salivary glands is adult. Age of onset can vary across affected individuals.