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familial nonchromaffin paraganglioma 4 (PGL4)
Epidemiology: rare
Pathology:
- mostly benign, highly vascular, slowly growing tumors in the head & neck
- carotid body is the most common site of the tumors (see carotid body tumor)
Genetics:
- autosomal dominant non-chromaffin paraganglioma type 4 associated with defect in SDHB
Database Correlations
OMIM 115310
References
OMIM :accession 605373