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

More general terms

• familial nonchromaffin paraganglioma

Database

• OMIM: https://mirror.omim.org/entry/115310