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Definition

A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Most commonly, they are located in the head and neck region, specifically at the carotid bifurcation, the jugular foramen, the vagal nerve, and in the middle ear.

Acronym

PGL4

Synonyms

Carotid body tumors and multiple extraadrenal pheochromocytomas
Familial chromaffin paraganglioma 4
Paraganglioma familial malignant
Paragangliomas hereditary extraadrenal
Pheochromocytoma extraadrenal and cervical paraganglioma
Pheochromocytoma familial extraadrenal

 
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