A paraganglioma is a tumour of neuroendocrine origin that arises in the paraganglia outside the adrenal gland. As in the rest of the body, they can produce hormones in the head and neck area, but this is rare. The tumour is very vascular and usually slow growing. There may be a hereditary component, so this should be investigated, and there is a small risk that the tumour will turn out to be malignant (malignant) and then have the ability to metastasise (spread). Historically, these tumours have often been referred to as a 'glomus tumour' (glomus tympanicum, glomus jugulare, glomus vagale, glomus caroticum), and may also be referred to as a chemodectoma.  

A working diagnosis or clinical question regarding a glomus tumor or paraganglioma in the head and neck region may arise during in the investigation of unclear resistance (nodule) on the neck, loss of cranial nerve function, pain, pressure symptoms of growing tumour, or depending on the tumour location symptoms such as pulse-synchronous tinnitus (ear buzzing), dysphagia (difficulty swallowing), and in rare cases dizziness, fainting, heart rhythm disorders or stroke. The question may also arise in connection with the investigation of hereditary cases. However, suspected paraganglioma are often results of an incidental finding in radiological investigations of other conditions.

Organisation and consultation process

At Uppsala University Hospital, assessment and treatment of paragangliomas in the head and neck area is multidisciplinary. The primary investigative service is ear, nose and throat (ENT), which is also a referral centre.

After initial assessment at the local hospital, treatment options are discussed at a multidisciplinary conference ('glomus MDK') involving specialists in vascular surgery, ENT specialists in tumour surgery, ENT specialists in otosurgery, oncology, endocrinology, radiology and pathology. Usually, the patient is also offered a personal visit to discuss treatment and make a treatment decision directly related to 'glomus MDK'. Patients scheduled for surgery are also offered a contact nurse in the Ear, Nose and Throat Department.

Contact us

As a care provider, do you have questions or want to discuss assessment, treatment or follow-up? In the first instance, contact a consultant from the tumour section at the Ear, Nose and Throat Department on 018-611 28 70.

Investigation prior to referral

Much of the assessment and preparation needed for the 'glomus MDK' assessment can be done through the local hospital. Exceptions to this may be PET scans and angiography with assessment of preoperative interventional radiology (embolisation) options.

Usually, CT and MRI scans with contrast agents administered in the venous and arterial phase (CT angio + MRI or MRI angio + CT) are needed. In the case of tumours involving the auditory system, audiometry should be performed and samples taken prior to treatment to determine whether the tumour is hormone-producing. The latter is a blood sample (s-methoxyadrenaline, s-methoxynoradrenaline / s-methoxycatecholamines) taken fasting after rest and with proximity to the chemistry lab as it should be kept on ice until analysis can be performed. Furthermore, testing for genetic variants (heredity) is recommended. The latter can be taken at Uppsala University Hospital for those patients who are on a personal visit, otherwise special referral is sent to clinical genetics.

Together with Skåne University Hospital, which is the country's second NHV unit, Uppsala University Hospital has common guidelines for referrals.

Checklista inför remittering av patient (Checklist for patient referral), in Swedish (pdf)

Riktlinjer för remittering (Guidelines for referral), in Swedish (cancercentrum.se)  

Treatment

The treatment modalities mainly discussed are surgery (which in turn may need to be preceded by interventional radiology) or radiation therapy . Many times, the therapy recommendation may be partly influenced by the patient's age and co-morbidities, but age alone is never a determining factor.

When surgery is required, it is performed at Uppsala University Hospital. Surgery for paragangliomas located on the neck always involves ENT specialists with a specialisation in tumour surgery and a vascular surgeon, and other specialists, such as a neurosurgeon, plastic surgeon or oral surgeon, are involved if necessary. In the case of paragangliomas located in the middle ear and base of the skull, the patient is operated on by ENT specialists with an otosurgical specialisation. Other specialists can also be involved in these cases, if necessary.

If radiation therapy is recommended, this is carried out and planned via the patient's home clinic, if necessary in consultation with an oncology specialist at Uppsala University Hospital.

In some cases, active excision ("wait and scan") is also recommended as the tumour is often slow-growing and treatment risks causing loss of function. In these cases, we recommend that follow-up radiological checks be reassessed at "glomus-MDK".

Treatment is based on the national treatment programme for adrenal tumours.

Follow-up

Follow-up after diagnosis of paraganglioma of the head and neck is individualised.

Surgical patients without a hereditary component are usually checked radiologically after about one year and only followed up thereafter if there are functional problems after surgery.

Radiation therapy cases are monitored with follow-up radiological examinations.

In the event of a wait-and-see approach, one or more radiological checks are usually performed.

If a hereditary variant of paraganglioma is found, the patient should be offered genetic counselling via clinical genetics, as well as additional testing and monitoring via a specialist in endocrine oncology. Where appropriate, this will be recommended after discussion at the 'glomus MDK', but referral is by the local hospital.