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Definition

A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. PGL1 is a rare autosomal dominant disorder which is characterized by the development of mostly benign, highly vascular, slowly growing tumors in the head and neck. In the head and neck region, the carotid body is the largest of all paraganglia and is also the most common site of the tumors.

Acronym

PGL1

Synonyms

Carotid body tumors
CBT1
Chemodectomas
Familial non-chromaffin paragangliomas 1
Familial paragangliomas non-chromaffin 1 with or without deafness
Glomus jugulare tumors
Glomus tumors familial 1
Paraganglioma carotid body
Paragangliomas familial 1
Paragangliomata
PGL

 
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