- UTHealth Houston Skull Base Program
- Paragangliomas
Paragangliomas
What are paragangliomas?
Paragangliomas are rare, typically slow-growing tumors that arise from paraganglia, or clusters of neuroendocrine cells often located near nerves and blood vessels. These tumors most commonly occur in the head and neck along the skull base, including areas such as the carotid body and jugular foramen. Some paragangliomas produce hormones that can affect blood pressure and heart rate, while others are nonfunctional. At UTHealth Houston Neurosciences, care is tailored based on tumor location, behavior, and overall patient health.
Causes
The exact cause of paragangliomas is not always known, but a significant number are associated with inherited genetic mutations. Conditions, such as hereditary paraganglioma-pheochromocytoma syndromes, often involving SDH gene mutations, can increase risk. Patients with a family history of these tumors or related syndromes may be more likely to develop paragangliomas, and genetic counseling may be recommended.
What are the symptoms of paragangliomas?
Symptoms depend on the tumor’s size, location, and whether it secretes hormones. Head and neck paragangliomas may cause a painless neck mass, hearing loss, pulsatile tinnitus, difficulty swallowing, hoarseness, or cranial nerve dysfunction. Functional tumors can lead to episodic high blood pressure, headaches, palpitations, sweating, and anxiety. Some tumors are discovered incidentally on imaging studies performed for unrelated reasons.
Diagnosis
Diagnosis typically involves an MRI or CT scan to define the tumor’s size and location. Functional imaging, such as a PET scan, may be used to evaluate metabolic activity or detect additional tumors. Blood and urine tests can help determine whether the tumor is producing hormones. Biopsy may be avoided due to the tumor’s vascular nature and bleeding risk.
How are paragangliomas treated?
Treatment depends on tumor size, location, growth rate, and whether it is functional. Observation with periodic imaging may be appropriate for small, asymptomatic tumors. Surgical removal is often recommended for accessible tumors causing symptoms or demonstrating growth. Radiation therapy, including stereotactic radiosurgery, may be used as a primary treatment or after surgery. Preoperative embolization may reduce blood flow to the tumor and lower surgical risk. For hormone-secreting tumors, medications are used to control blood pressure and prevent complications. Many patients experience good long-term outcomes, particularly with early detection and appropriate management. Because some paragangliomas grow slowly or may recur, long-term follow-up with imaging and clinical evaluation is essential. Patients with hereditary forms may require ongoing surveillance for additional tumors.