PHEOCHROMOCYTOMA (Chromaffin 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 (4^th 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
• Complete tumor removal is the uhimate therapeutic goal, ,can be achieved by partial or total adrenalectomy.

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