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30 years old male with hypertension




  • This case shows: CT, T2W MRI, MIBG scintigraphy images.
  • Pheochromocytoma is a subtype of Paraganglioma, a neuroendocrine tumor that arises from paraganglioma tissue.
  • Rule of ā€œ10sā€: 10% of pheochromocytomas are extra-adrenal, 10% are bilateral, and 10% are malignant.
  • Most common location of extra-adrenal pheochromocytoma is the organ of Zuckerkandl, near the aorrtic bifurcation.
  • Paraganglioma cells belong to the amine precursor uptake and dexarboxylation (APUD) system. Cells may secrete catecholamines (epinephrine, dopamine, norepinephrine) or be nonfunctional.
  • Parasympathetic paragangliomas include the Glomus tumors (tympanicum, jugulare, vagale) and carotid body tumors.
  • Clinical picture: Episodic(50%) or sustained (50%) hypertension, tachycardia, diaphoresis, headeche.
  • Elevated VMA  in 24-hour urine in 50%.
  • Elevated serum catecholamines urine metanephrines.
  • Associations: MEN in 5% (pheochromocytomas usually bilateral), Neurofibromatosis (10% have pheochromocytomas), Von Hippel-Lindau disease (10%have), Familial pheochromocytosis (10% of all pheochromocytomas).
  • Radiographic features: Adrenal mass, strong contrast enhancement (CT or angiography), Calcification, MRI demonstrates very high signal on T2W.
  • Percutaneous biopsy is method of choice for tissue diagnosis.

Metaiodobenzylguanidine (MIBG)

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