Head and Neck Paragangliomas

What is head and neck paragangliomas?

Head and neck paragangliomas are rare tumors that grow from small clusters of nerve tissue called paraganglia. Paraganglia are part of the autonomic nervous system, the part of the nervous system that controls automatic body functions such as heart rate and blood pressure. In the head and neck, these clusters sit close to major blood vessels and nerves, especially around the carotid arteries in the neck, the middle ear, the jugular vein at the base of the skull, and the vagus nerve, which runs from the brain into the chest.

Most head and neck paragangliomas are benign, meaning they are not cancer and do not spread to other parts of the body. They usually grow slowly, often over many years. Because of where they sit, however, even a benign tumor can press on nearby nerves, blood vessels, or the structures of the ear and cause problems. A small proportion behave in a malignant way, which doctors define as the tumor spreading to lymph nodes or distant organs.

Doctors often name these tumors by location:

• Carotid body tumor – at the point in the neck where the main carotid artery divides in two.
• Jugular paraganglioma (sometimes called glomus jugulare) – near the jugular vein at the base of the skull.
• Tympanic paraganglioma (glomus tympanicum) – within the middle ear.
• Vagal paraganglioma – along the vagus nerve in the upper neck.
• Laryngeal paraganglioma – in the voice box, which is very rare.

These tumors are most often found in adults, commonly in middle age, although inherited forms may appear at a younger age. They are diagnosed slightly more often in women for some locations. Head and neck paragangliomas are related to tumors called pheochromocytomas, which arise in the adrenal glands, and to paragangliomas in the chest and abdomen. Unlike those tumors, head and neck paragangliomas only rarely release stress hormones (catecholamines) into the bloodstream.

Head and neck paragangliomas symptoms

Head and neck paragangliomas symptoms depend largely on where the tumor sits and how large it has become. Many tumors cause no symptoms for years and are found by chance on a scan done for another reason. When symptoms do appear, they tend to develop slowly.

• A painless lump in the side of the neck that slowly enlarges
• Pulsatile tinnitus, a rhythmic whooshing or thumping sound in one ear that beats in time with the pulse
• Hearing loss in one ear, or a feeling of fullness or pressure in the ear
• Hoarseness or a weak, breathy voice
• Difficulty swallowing or a sensation of food catching in the throat
• Drooping of one shoulder or weakness turning the head
• Weakness or deviation of the tongue to one side
• Dizziness or unsteadiness
• Rarely, episodes of rapid heartbeat, sweating, headache, flushing, or high blood pressure

Carotid body tumors most often present as a slow-growing lump in the upper neck that may move from side to side but not up and down. It is usually painless, and some people notice a pulsing feeling in it.

Jugular and tympanic paragangliomas mainly affect the ear. Pulsatile tinnitus and hearing loss are the most common early complaints. A doctor examining the ear may see a reddish mass behind the eardrum. As a jugular tumor enlarges, it may affect the nerves that pass through the base of the skull, leading to hoarseness, swallowing difficulty, shoulder weakness, or facial weakness.

Vagal paragangliomas often cause a neck lump higher up than a carotid body tumor and are more likely to affect the voice and swallowing early, because the vagus nerve controls muscles in the throat and voice box.

In a small number of cases, the tumor releases catecholamines, the same hormones the body makes during stress. This may cause spells of palpitations, sweating, pounding headache, anxiety, or high blood pressure. These symptoms are uncommon in head and neck paragangliomas but are important to recognize because they change how the tumor is managed.

Causes and risk factors

The exact reason a paraganglioma starts to grow is not fully understood. Head and neck paragangliomas causes are best understood in two groups: inherited (genetic) causes and sporadic tumors that develop without a known family pattern.

A substantial proportion of people with these tumors carry an inherited change (mutation) in one of a group of genes that help cells use oxygen and produce energy. The best known are the succinate dehydrogenase genes, often written as SDHD, SDHB, SDHC, SDHA, and SDHAF2. Changes in these genes are passed down in families and increase the chance of developing paragangliomas in the head and neck and elsewhere in the body, sometimes more than one tumor over a lifetime. Because these gene changes can be present even when no relative has had a known tumor, doctors commonly recommend genetic counseling and testing for anyone diagnosed with a paraganglioma.

Other, less common inherited conditions can also be associated with paragangliomas, including certain syndromes that affect several glands and organs.

Chronic low oxygen levels appear to play a role in some cases. Carotid body tumors are reported more often in people who live for long periods at high altitude and in people with long-standing lung or heart conditions that lower blood oxygen. The carotid body normally senses oxygen levels, and prolonged stimulation is thought to encourage its cells to multiply.

Recognized risk factors include:

• A known inherited mutation in an SDH gene or related gene
• A family history of paraganglioma or pheochromocytoma
• A previous paraganglioma anywhere in the body
• Long-term residence at high altitude
• Chronic conditions that cause low blood oxygen
• Female sex, for some tumor locations

There is no evidence that diet, lifestyle habits, or infections cause these tumors, and nothing a person did or did not do is thought to bring them on.

Head and neck paragangliomas diagnosis

Head and neck paragangliomas diagnosis usually begins when a doctor notices a neck lump, an unusual finding in the ear, or hears about pulsatile tinnitus or hearing loss. Because these tumors are richly supplied with blood vessels, doctors generally avoid taking a needle or surgical biopsy, which could cause heavy bleeding. Instead, the diagnosis rests mainly on imaging, blood or urine tests, and the typical appearance of the tumor.

Tests your doctor may use include:

• Physical examination – feeling the neck, looking into the ear with a microscope or scope, checking hearing, and testing the function of the nerves that control the face, tongue, voice, shoulder, and swallowing.
• Hearing tests (audiometry) – to measure the type and degree of any hearing loss.
• Ultrasound with Doppler – often the first scan for a neck lump, showing a mass with a rich blood supply near the carotid artery.
• Computed tomography (CT) – a detailed X-ray scan that shows how the tumor relates to bone, particularly the skull base and middle ear.
• Magnetic resonance imaging (MRI) – a scan using magnets and radio waves that shows the tumor, its blood vessels, and nearby soft tissues in fine detail. Paragangliomas often have a characteristic speckled appearance on MRI.
• Angiography – an X-ray study of the blood vessels, sometimes used to map the tumor’s blood supply before surgery.
• Functional (nuclear medicine) imaging – whole-body scans using a small amount of a radioactive tracer that is taken up by paraganglioma cells. These scans help find additional tumors elsewhere in the body, which is especially important in people with inherited forms.
• Blood or urine tests for catecholamines and their breakdown products (metanephrines) – to check whether the tumor is releasing hormones. This is generally recommended for everyone diagnosed with a paraganglioma.
• Genetic testing – a blood or saliva test to look for inherited gene changes, usually offered with genetic counseling.

Imaging also allows doctors to classify the tumor by size and how far it extends, for example whether a jugular tumor has grown into the bone of the skull base or toward the brain. This staging guides treatment planning. Because the tumor can affect several specialties, care is often shared between ear, nose, and throat surgeons, skull base surgeons, radiation oncologists, endocrinologists, radiologists, and geneticists.

Head and neck paragangliomas treatment

Head and neck paragangliomas treatment is individualized. There is no single right approach, and the recommendation depends on the tumor’s location and size, whether it is growing, whether it is producing hormones, the person’s age and general health, existing nerve damage, and personal preference. The main goal is to control the tumor while preserving nerve function and quality of life as far as possible.

Active surveillance (watchful waiting). Because many of these tumors grow very slowly, doctors often recommend monitoring rather than immediate treatment, particularly for small tumors, tumors that are not causing symptoms, and older adults or people with other health conditions. This involves repeated MRI or CT scans at intervals to check for growth, along with regular hearing and nerve checks. Treatment is considered if the tumor grows or symptoms develop.

Surgery. Surgical removal can potentially remove the tumor completely and is often considered for carotid body tumors, tympanic tumors confined to the middle ear, and some larger or growing tumors in younger, healthy people. Surgery near the skull base and major nerves is complex. Possible complications include bleeding, stroke, and permanent damage to the nerves that control swallowing, voice, the tongue, the shoulder, or the face. Before surgery, an interventional radiologist may block the tumor’s blood vessels (embolization) to reduce bleeding. If the tumor produces catecholamines, medication to control blood pressure and heart rate is started for a period before the operation.

Radiation therapy. Focused radiation, either delivered in a single high-dose session (stereotactic radiosurgery) or in smaller daily doses over several weeks, aims to stop the tumor from growing rather than remove it. It is frequently used for jugular and vagal tumors, where surgery carries a high risk of nerve injury, and for people who are not good candidates for surgery. Tumor control rates are generally reported to be favorable, though the tumor usually remains visible on scans afterward and lifelong follow-up is still needed. Side effects can include fatigue, skin irritation, and, less commonly, later effects on hearing or nearby tissues.

Medication. Drugs do not shrink most head and neck paragangliomas. They are mainly used to control blood pressure and heart rate in the uncommon hormone-producing tumors. For the rare tumors that have spread, options such as targeted radioactive drugs, chemotherapy, or other systemic therapies may be discussed by a specialist team.

Rehabilitation and supportive care. When the tumor or its treatment affects nerve function, rehabilitation is an important part of care. This may include swallowing therapy with a speech-language pathologist, voice therapy or procedures to improve a weak vocal cord, hearing aids or other hearing devices, and physical therapy for shoulder weakness. Support for tinnitus and balance problems may also be offered.

Within Acibadem, head and neck paragangliomas are generally assessed and managed through the Otorhinolaryngology (ENT) department, working together with other specialties as needed.

Living with head and neck paragangliomas and outlook

For most people, the outlook with a head and neck paraganglioma is reassuring in the sense that the tumor is usually benign and slow growing. Many people live for decades with a stable tumor under observation, or with a tumor that has been removed or controlled with radiation. The main long-term concerns are the effects of the tumor or its treatment on nerve function and hearing, and the small chance that the tumor recurs, grows, or spreads.

Lifelong follow-up is generally recommended. Even after apparently complete removal, tumors can recur years later, and people with inherited gene changes can develop new tumors at other sites. Follow-up typically involves periodic imaging, hormone tests where relevant, and clinical checks. The interval between visits is usually set by your care team based on your situation.

Adjusting to permanent changes in voice, swallowing, or hearing can take time. Many people find that structured rehabilitation, patience, and practical adaptations make a meaningful difference. Living with uncertainty about a tumor that is being watched rather than treated can also be stressful, and talking with your care team, a counselor, or a patient support group may help.

If genetic testing shows an inherited mutation, close relatives may be offered testing so that any tumors can be found early, when they are small and easier to manage. Genetic counselors can help families understand what a result means and what surveillance may be advisable.

Frequently asked questions

Are head and neck paragangliomas cancer?

In most cases, no. The large majority of head and neck paragangliomas are benign and do not spread. Doctors cannot always tell from the appearance of the tumor whether it will behave in a malignant way; a tumor is generally considered malignant only if it spreads to lymph nodes or distant organs. Certain inherited gene changes are associated with a higher chance of spread, which is one reason genetic testing is often recommended.

What are the first symptoms of head and neck paragangliomas?

Early head and neck paragangliomas symptoms are often subtle or absent. The most common first signs are a slowly enlarging painless lump in the neck, or a pulsing sound in one ear with gradual hearing loss. Some tumors are discovered by chance on a scan done for an unrelated reason. Nerve-related symptoms such as hoarseness or swallowing difficulty tend to appear later as the tumor grows.

What causes head and neck paragangliomas?

Head and neck paragangliomas causes include inherited changes in genes such as SDHD and SDHB, which are found in a significant proportion of people with these tumors, as well as long-term exposure to low oxygen levels, for example from living at high altitude. In many people no clear cause is identified. They are not caused by lifestyle choices, infection, or injury.

How is head and neck paragangliomas diagnosis confirmed without a biopsy?

Because these tumors bleed easily, doctors usually avoid biopsy. The diagnosis is generally made from the typical appearance on MRI, CT, or ultrasound, combined with the tumor’s location and blood supply pattern. Blood or urine hormone tests and specialized nuclear medicine scans add further confidence and help check for additional tumors elsewhere. In practice, this combination is usually enough to plan treatment.

Do head and neck paragangliomas always need treatment?

Not always. Because many grow very slowly, observation with regular scans is a recognized and common approach, especially for small tumors that are not causing symptoms. Your doctor may recommend treatment if the tumor is growing, causing symptoms, producing hormones, or threatening important structures. The decision balances the risks of the tumor against the risks of treatment.

What is the best head and neck paragangliomas treatment: surgery or radiation?

Neither is best for everyone. Surgery offers the possibility of removing the tumor entirely but carries a risk of nerve injury, particularly at the skull base. Radiation aims to stop growth with a lower risk to nerves but leaves the tumor in place and requires ongoing monitoring. The choice depends on tumor location and size, age, overall health, existing nerve function, and personal preference, and is usually made with a multidisciplinary team.

Can head and neck paragangliomas come back after treatment?

Yes, recurrence is possible even after apparently complete surgical removal, sometimes many years later, and people with inherited forms can develop new tumors at other sites. For this reason, long-term follow-up with periodic imaging is generally recommended, and the schedule is tailored by your care team.

When to see a doctor

Make an appointment with a doctor if you notice a lump in your neck that persists for more than a few weeks, a whooshing or pulsing sound in one ear, hearing loss in one ear, ongoing hoarseness, or new difficulty swallowing. These symptoms have many possible causes, and most are not paragangliomas, but they should be evaluated.

Seek urgent medical care if you experience any of the following red-flag warning signs:

• Sudden severe headache with a very fast or pounding heartbeat, sweating, and very high blood pressure
• Sudden weakness or numbness of the face, arm, or leg, trouble speaking, or loss of vision, which may indicate a stroke
• Difficulty breathing or noisy breathing
• Choking or inability to swallow liquids or saliva
• Heavy bleeding from the ear or nose
• Sudden complete hearing loss in one ear or sudden severe dizziness
• A neck lump that grows rapidly over days or becomes painful, red, or hot

If you have already been diagnosed with a head and neck paraganglioma and develop new nerve symptoms, such as changes in voice, swallowing, shoulder strength, tongue movement, or facial movement, tell your care team promptly, as these may indicate tumor growth and can change the treatment plan.