Paragangliomas are almost always benign (non-cancerous) and are usually very slow growing.
Most paragangliomas are not caused by inheriting faulty genes but occur by chance (these are known as sporadic tumours). In paragangliomas affecting the head and neck about 1 in 25 people with a single tumour, and as many as 1 in 3 people with multiple tumours, have inherited a faulty gene.
Very occasionally paragangliomas in other parts of the body may be linked to rare inherited conditions. These include:
- Neurofibromatosis type 1 (NF1). This affects about 1 in 3000 people. It causes coffee coloured patches on the skin and benign tumours of the nerves.
- Von Hippel Lindau syndrome affects about 1 in 85,000 people.
- Carney's triad: a very rare condition where paragangliomas occur along with other benign tumours of the lung and stomach.
References
- Pheochromocytoma: the Expanding Genetic Differential Diagnosis Bryant J et al. JNCI 95(16) 2003
- Evolving concepts in pheochromocytoma and paraganglioma. Dahia P. Current Opinion in Oncology 18:1-8 2006
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