Overview
Nasopharyngeal teratoma is a rare congenital germ cell tumor that arises in the nasopharynx (the upper part of the throat behind the nose). Teratomas are tumors composed of tissues derived from all three embryonic germ layers — ectoderm, mesoderm, and endoderm — and may contain elements such as skin, hair, teeth, bone, cartilage, and neural tissue. When located in the nasopharynx, these tumors are also sometimes referred to as epignathus when they protrude from the oral cavity or palate, though nasopharyngeal teratoma more specifically refers to masses arising within or projecting into the nasopharyngeal space. The ICD-10 classification D10.6 indicates a benign neoplasm of the nasopharynx. Nasopharyngeal teratomas are typically diagnosed at birth or prenatally via ultrasound, as they can cause significant airway obstruction, feeding difficulties, and respiratory distress in neonates. Key clinical features include nasal obstruction, difficulty breathing, inability to feed, and a visible or palpable mass in the oral cavity or nasopharynx. In severe cases, large tumors detected prenatally may cause polyhydramnios (excess amniotic fluid) due to impaired fetal swallowing. The condition primarily affects the upper aerodigestive tract and can secondarily impact respiratory and nutritional status. Treatment is primarily surgical excision, which is often curative for mature (benign) teratomas. The surgical approach depends on the size and extent of the tumor and may range from transoral resection to more complex craniofacial procedures. Most nasopharyngeal teratomas are histologically mature and benign, carrying an excellent prognosis after complete surgical removal. However, careful histological examination is essential to rule out immature or malignant components, which would require additional treatment. Prenatal planning, including the EXIT (Ex Utero Intrapartum Treatment) procedure to secure the airway during delivery, may be necessary for large tumors identified before birth.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Nasopharyngeal teratoma.
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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 Nasopharyngeal teratoma.
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Common questions about Nasopharyngeal teratoma
What is Nasopharyngeal teratoma?
Nasopharyngeal teratoma is a rare congenital germ cell tumor that arises in the nasopharynx (the upper part of the throat behind the nose). Teratomas are tumors composed of tissues derived from all three embryonic germ layers — ectoderm, mesoderm, and endoderm — and may contain elements such as skin, hair, teeth, bone, cartilage, and neural tissue. When located in the nasopharynx, these tumors are also sometimes referred to as epignathus when they protrude from the oral cavity or palate, though nasopharyngeal teratoma more specifically refers to masses arising within or projecting into the nas
How is Nasopharyngeal teratoma inherited?
Nasopharyngeal teratoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Nasopharyngeal teratoma typically begin?
Typical onset of Nasopharyngeal teratoma is neonatal. Age of onset can vary across affected individuals.
Which specialists treat Nasopharyngeal teratoma?
2 specialists and care centers treating Nasopharyngeal teratoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.