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phaeochromocytoma

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phaeochromocytoma

Introduction

  • pheochromocytomas arise from chromaffin cells and which secrete catecholamines
  • they are rare tumours, arising in only 2-8 people per million population
  • 85% arise from the adrenal (11% are malignant), 15% arise from extra-adrenal sources such as sympathetic ganglia (30% are malignant)
  • accounts for ~10% of adrenal masses and 10% of cases are bilateral - see adrenal tumours
  • 15–20% are hereditary
  • mostly occurs in young or middle age adults
  • they were 1st described in 1886 by Felix Fränkel, and the name was coined by Ludwig Pick in 1912

Diagnosis

  • often evident from clinical picture:
    • episodic hypertension, headache, sweating, palpitations, anxiety, pallor and tremors
    • may be associated with endocrine syndromes such as:
      • neurofibromatosis
      • von Hippel-Lindau disease (retinal angiomas) - this accounts for 5% of cases
      • multiple endocrine neoplasm II (medullary thyroid carcinoma)
  • easy to detect on MRI
  • diagnostic tests include:
    • plasma catecholamines (>2000 pg/mL) - lower levels may require further Ix such as a clonidine suppression test
    • 24hr urinary metanephrines >1.6 mg/24 hr
      • NB. metamphetamine (ice) users may also have high urinary metanephrine levels and thus should have a week of abstinence before re-testing

DDx

Rx

  • Rx is usually surgical resection but there is a considerable risk of profound hypotension post-op which is usually prevented by measures such as pre-op salt loading
  • 3% recur
  • patients have a relative risk of 3-4x of secondary cancers
phaeochromocytoma.txt · Last modified: 2020/10/07 12:25 by gary1