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WHO Classification of Tumours
Adrenal gland


Extra-adrenal paraganglioma: Carotid body, jugulotympanic, vagal, laryngeal, aortico-pulmonary tumours arising from the paraganglia distributed along the parasympathetic nerves in the head, neck and mediastinum. These tumours are histologically identical and are named by anatomical site of origin
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Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004


These neoplasms are rare. Jugulotympanic and carotid body tumours are the two most frequent. A significant increase in the incidence of carotid body paraganglioma has been reported in humans dwelling at high altitude, where a marked female predilection has been reported

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Rodríguez-Cuevas S, López-Garza J, Labastida-Almendaro S (1998)
Carotid body tumors in inhabitants of altitudes higher than 2000 meters above sea level.
Head Neck 20: 374-8

It is generally accepted that a paraganglioma is determined to be malignant only when metastases are demonstrated.