Pheochromocytoma

Pheochromocytoma
Other namesPhaeochromocytoma, adrenal medullary tumor, Chromaffin Cell Tumors, Paraganglioma
Normal remnant adrenal gland (left) with a pheochromocytoma (right) involving the adrenal medulla
Pronunciation
  • /ˌfˌkrmsʌɪˈtmə/ (fee-oh-kroh-moh-sahy-toh-muh)
SpecialtyEndocrinology, oncology
SymptomsHypertension, tachycardia, sweating, headache, pallor
ComplicationsHypertensive crisis
Causes80% Genetic
Diagnostic methodElevated plasma free metanephrines, plasma catecholamines, urinary catecholamines, MRI, PET Scan
TreatmentSurgery, chemotherapy, radiation, and pharmacologic agents
MedicationAlpha blocker Doxazosin Prazosin Terazosin
Frequency0.8 per 100,000 person-years

Pheochromocytoma (British English: phaeochromocytoma) is a rare tumor of the adrenal medulla composed of chromaffin cells and is part of the paraganglioma (PGL) family of tumors, being defined as an intra-adrenal PGL. These neuroendocrine tumors can be sympathetic, where they release catecholamines into the bloodstream which cause the most common symptoms, including hypertension (high blood pressure), tachycardia (fast heart rate), sweating, and headaches. Some PGLs may secrete little to no catecholamines, or only secrete paroxysmally (episodically), and other than secretions, PGLs can still become clinically relevant through other secretions or mass effect (most common with head and neck PGL). PGLs of the head and neck are typically parasympathetic and their sympathetic counterparts are predominantly located in the abdomen and pelvis, particularly concentrated at the organ of Zuckerkandl at the bifurcation of the aorta.