familial nonchromaffin paraganglioma 1 (PGL1)

Epidemiology

rare

Pathology

• mostly benign, highly vascular, slowly growing tumors in the head & neck
• associated_neoplasm[s]
  • paraganglioma hemodectoma carotid body tumor glomus jugular tumor
• carotid body is the most common site of the tumors (see carotid body tumor)

Genetics

• autosomal dominant non-chromaffin paraganglioma type 1 associated with defects in SDHD (penetrance is incomplete when transmitted through fathers; not transmitted maternally)

More general terms

• familial nonchromaffin paraganglioma

Database

• OMIM: https://mirror.omim.org/entry/168000
• OMIM: https://mirror.omim.org/entry/602690