Paragangliomas, sometimes called glomus tumours, are slow growing tumours arising from non-chromaffin paraganglion cells that are scattered throughout the body from the base of skull to the urinary bladder.
Paragangliomas can occur from the base of skull to the pelvis, anywhere there are paraganglia, and are divided according to their location. These are discussed separately, as their imaging features, clinical presentation and treatment are to some degree location dependent.
- base of skull and neck (see paragangliomas of the head and neck )
- mediastinal (see mediastinal paraganglioma)
- paravertebral (aortosympathetic paraganglia)
- great vessels of the chest (aortopulmonary paraganglia)
- cardiac (extremely rare; may be located along the epicardium, in the atrial cavity, the interatrial septum or the ventricles) 9
- spinal cord, usually cauda equina (see spinal paraganglioma)
All paragangliomas consist two types of cells; type I and type II. The main components are lobules or nests of chief cells (type I); these structures are known as Zellballen. They are surrounded by a single layer of sustentacular cells (type II) 8.
Immunohistochemical examination confirms neuroendocrine differentiation of chief cells (type I) 5:
Sustentacular cells (type II):
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