Hormonal Syndromes Factsheet

Pheochromocytoma

Pheochromocytomas (PH) are rare NETs which start in the adrenal glands on top of the kidneys. Most produce excessive amounts of hormones resulting in symptoms such as:

• high blood pressure and rapid heart rate (palpitations)
• sweating
• severe headaches
• anxiety and feelings of rapid heart rate
• loss of weight

The remainder have no symptoms and are often undiagnosed for many years. They mostly affect adults however can also affect children and adolescents.

Paraganglioma

Paraganglia are groups of cells found near nerve cell bundles called ganglia. These ganglia are located in the head, neck, thorax, abdomen or pelvis and are classified as either parasympathetic or sympathetic, depending on which nerves they are associated with. A tumour involving the paraganglia is known as a paraganglioma.

• Parasympathetic paragangliomas are mainly found in the head and neck, usually do not secrete hormones and rarely metastasize.
• Sympathetic paraganglioma are found in the thorax, abdomen and pelvis, secrete hormones such as adrenaline or noradrenaline, and metastasize in one in five cases.

More than a third of patients with paraganglioma have inherited mutations.

The main treatment modalities are surgery, embolization, radiation therapy and stereotactic radiosurgery. People with these NETs may also be offered chemotherapy and peptide receptor radionuclide therapy (PRRT).