Paraganglioma and Phaeochromocytoma

Paragangliomas are rare tumours that grow from nerve-related cells found along the spine, in the abdomen and chest, and in the head and neck, for example at the carotid artery or near the ear. When the same type of tumour arises inside the adrenal gland it is called a phaeochromocytoma.

Most are benign, but they matter for two reasons. Many release adrenaline-like hormones that cause dangerous surges in blood pressure, and a minority spread. Up to 4 in 10 are linked to an inherited gene change, so every patient is offered genetic testing.

Symptoms

• Attacks of pounding headache, sweating and a racing heart
• High blood pressure that is episodic or hard to control
• Paleness, anxiety or tremor during attacks
• A painless lump in the neck
• Pulsating noise in one ear or hearing loss
• Hoarseness or swallowing difficulty with skull base tumours
• No symptoms at all, found on a scan

Causes and risk factors

The tumours develop from paraganglia, small clusters of cells of the autonomic nervous system. Those in the abdomen and chest usually secrete noradrenaline or adrenaline, while most head and neck paragangliomas do not. Inherited changes in the SDHB, SDHD, SDHC, VHL, RET and NF1 genes account for a large share. SDHB changes carry the highest risk of spread. Living for many years at high altitude is a recognised factor for carotid body tumours.

How it is diagnosed

• Plasma or 24-hour urine metanephrines: The key hormone test. It must be done before any biopsy or surgery.
• CT or MRI: Locates the tumour and shows its relation to blood vessels and nerves.
• Functional imaging: Gallium-68 DOTATATE PET-CT, or MIBG and FDG PET, to look for multiple or metastatic tumours.
• Genetic testing: Offered to all patients, since a result changes follow-up and screening of relatives.
• No needle biopsy: Biopsy of an unprepared secreting tumour can trigger a hypertensive crisis and is avoided.

Treatment options

• Pre-operative blockade: Alpha blockers such as doxazosin or phenoxybenzamine for 1 to 2 weeks, with fluids and salt and then a beta blocker if needed, make surgery safe.
• Surgical removal: Laparoscopic adrenalectomy for phaeochromocytoma, open or minimally invasive removal for abdominal tumours, and neck or skull base surgery for head and neck tumours.
• Embolisation before surgery: Reduces bleeding from large carotid body or jugular tumours.
• Radiotherapy or radiosurgery: Controls growth of skull base tumours where surgery would risk the nerves of swallowing and voice.
• Observation: Small, non-secreting head and neck tumours in older people often grow very slowly and can be watched.
• Treatment of metastatic disease: Radionuclide therapy with lutetium DOTATATE or MIBG, chemotherapy or targeted drugs in specialist centres.

When it is urgent

A severe headache with very high blood pressure, chest pain, breathlessness, palpitations or confusion may be a catecholamine crisis. Call emergency services and tell them the diagnosis. Such a crisis can be set off by anaesthesia, some medicines and contrast procedures when the tumour is not blocked.

Travelling to Türkiye for treatment

These tumours are rare enough that seeing a team with real volume, including endocrinology, experienced surgery, anaesthesia and nuclear medicine, is a sound reason to travel. Allow about 2 to 3 weeks if blockade is begun at home beforehand, which your own doctor should supervise. Genetic results affect your relatives and lifelong yearly metanephrine checks are needed, so arrange follow-up locally before you go.

Send your reports, scans and a short history and a Clinic-Y coordinator replies within 24 hours with suitable teams and written, all-inclusive proposals side by side. Reviewing your case is free.

Frequently Asked Questions