Pheochromocytoma

4.8
(16)
  • Neuroendocrine tumor
  • MC site: Adrenal medulla
  • MC site of extra-adrenal pheochromocytoma/ paraganglioma: Organ of Zuckerkandl
  • The organ of Zuckerkandl is a chromaffin body derived from neural crest located at the bifurcation of aorta or at the origin of inferior mesenteric artery

  • Rule of 10% of pheochromocytoma:
    1. 10% tumors are extra-adrenal
    2. 10% tumors are bilateral
    3. 10% tumors are malignant,
  • Remember: 25% tumors are familial.
  • MC symptom: Headache
  • MC presentation: Episodic hypertension
  • Screening test of choice: 24 hr urinary VMA estimation
  • Most sensitive test: Plasma free metanephrine
  • Imaging IOC: MRI → ‘Light bulb sign’

  • Most sensitive investigation for localisation of extra-adrenal pheochromocytoma: Fluorodopa PET scanning
  •  Markers:
    1. Synpatophysin
    2. Chromogranin
    3. S100
  • HPE: Zell Ballen Pattern – Nests of cells separated by the stroma

  • Most reliable feature of malignancy in pheochromocytoma: Metastasis
  • Medical therapy: 𝛼-blockade (phenoxybenzamine) followed by 𝛽-blockade
  • Surgery: Open adrenalectomy
  • DOC for intraoperative control of blood pressure in pheochromocytoma surgery: IV Phentolamine
  • Anesthetic agent which should not be used during surgery: Halothane → Sensitizes the myocardium to catecholamines → Leading to arrhythmia.

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