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paraganglioma

Etiology: - arise from specialized neural crest cells associated with autonomic ganglia throughout the body (paraganglion) - subtypes by anatomic site include: - carotid body paraganglioma (chemodectoma) - jugulotympanic paraganglioma (glomus jugulare tumor) - pheochromocytoma of adrenal gland Epidemiology: - 5-10% occur outside the adrenal gland - Can also occur in cauda equina region of spinal cord, (~120 cases reported as of 2000) Pathology: - generally benign, neural crest derived tumors of parasympathetic ganglia - highly vascular, slowly growing tumors in the head & neck - non-chromaffin paragangliomas generally carotid body tumor - associated hereditary neoplastic syndromes include: - Carney complex - Carney triad - multiple endocrine neoplasia (MEN2A & MEN2B) - neurofibromatosis 1 - von Hippel-Lindau disease - Sturge-Weber syndrome Genetics: - autosomal dominant non-chromaffin paraganglioma type 1 associated with defects in SDHD (penetrance is incomplete when transmitted through fathers; not transmitted maternally) - autosomal dominant non-chromaffin paraganglioma type 3 associated with defect in SDHC Laboratory: 1) 24 hour urine metanephrines* 2) 24 hour urine catecholamines 3) plasma free metanephrines* * urinary metanephrines with higher specificity * plasma free metanenephrines with higher sensitivity [3]

Related

paraganglion

Specific

benign paraganglioma carotid body tumor familial nonchromaffin paraganglioma glomus jugulare tumor; jugulotympanic paraganglioma pheochromocytoma; paroxysmal hypertension; adrenal medullary paraganglioma; chromoffinoma primary CNS paraganglioma; spinal cord paraganglioma

General

autonomic nervous system neoplasm neuroendocrine neoplasm

References

  1. Lenders JW, Duh QY, Eisenhofer G et al Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99(6):1915-42. PMID: 24893135
  2. http://www.pathologyoutlines.com/adrenal.html
  3. Medical Knowledge Self Assessment Program (MKSAP) 17. American College of Physicians, Philadelphia 2015