Pheochromocytoma

PHEOCHROMOCYTOMA


PHEOCHROMOCYTOMA (Chroffin tumour)

  • Pheochromocytoma & paraganglioma are catecholamines producing tumours derived from sympathetic and parasympathetic nervous system.
  • They are derived from Chromaffin cells.
  • Neuroendocrine cells of the adrenal medulla.
  • 90% of non familial cases are bilateral

Sites of pheochromocytoma

  • Adrenal gland (most common)
  • Head and Neck
  • Thorax (mediastinum)
  • Intra abdominal (paraaortic)
  • Bladder

Etiology-

  • Hypertension
  • Sporadic pheochromocytoma (4th decade)
  • Hereditary pheochromocytoma- MEN type 2, Van- Hippel Lindau (VHL) syndrome, neurofibromatous type-1.

Pathology-

  • The classic “rule of tens” for pheochromocytoma is-
  1. 10% bilateral
  2. 10% extra adrenal
  3. 10% malignant
  • Well defined nests (Zellballen pattern)

Clinical features-

  • Headache, palpitations & sweating- adrenal tumour
  • Hypertension (most common)
  • Congestive heart failure, MI, pulmonary oedema
  • Cerebral hemorrhage
  • Weight loss, Increased hematocrit, Impaired glucose tolerance

Investigations-

  • Confirmation of excessive catecholamines levels in the patients by measuring adrenaline & nor adrenaline.
  • MRI is preferred
  • Classically phaeochromocytoma shows ‘swiss cheese configuration’.
  • Urinary catecholamines and their metabolites  such as VMA and metanephrines.
  • MIBG
  • CT scan- localise

Treatment-

  • Laproscopic resection
  • Alpha adrenoreceptor blocker (phenoxybenzamine)- block catecholamine excess
  • Beta blockade- tachycardia or arrhythmias
  • Central venous catheter & invasive arterial monitoring used.
  • Adult Dose of Clonidine for Clonidine Suppression test is 0.3 mg (0.3mg/70kg) administered orally. Clonidine Suppression Test

Exam Important

Sites of pheochromocytoma

  • Adrenal gland (most common)
  • Head and Neck
  • Thorax (mediastinum)
  • Intra abdominal (paraaortic)
  • Bladder

Etiology-

  • Hypertension
  • Sporadic pheochromocytoma (4th decade)
  • Hereditary pheochromocytoma- MEN type 2, Van- Hippel Lindau (VHL) syndrome, neurofibromatous type-1.

Pathology-

  • The classic “rule of tens” for pheochromocytoma is-
  1. 10% bilateral
  2. 10% extra adrenal
  3. 10% malignant
  • Well defined nests (Zellballen pattern)

Clinical features-

  • Headache, palpitations & sweating- adrenal tumour
  • Hypertension (most common)
  • Congestive heart failure, MI, pulmonary oedema
  • Cerebral hemorrhage
  • Weight loss, Increased hematocrit, Impaired glucose tolerance

Investigations-

  • Confirmation of excessive catecholamines levels in the patients by measuring adrenaline & nor adrenaline.
  • MRI is preferred
  • Classically phaeochromocytoma shows ‘swiss cheese configuration’.
  • Urinary catecholamines and their metabolites  such as VMA and metanephrines.
  • MIBG
  • CT scan- localise

Treatment-

  • Laproscopic resection
  • Alpha adrenoreceptor blocker (phenoxybenzamine)- block catecholamine excess
  • Beta blockade- tachycardia or arrhythmias
  • Central venous catheter & invasive arterial monitoring used.
  • Adult Dose of Clonidine for Clonidine Suppression test is 0.3 mg (0.3mg/70kg) administered orally. Clonidine Suppression Test
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