Pheochromocytoma Clinical Features Diagnosis Treatment

All about Pheochromocytoma Clinical Features Diagnosis Treatment

Definition

A tumor derived from neural crest cells of the sympathetic nervous system that is responsible for about 0.1% to 2% of all cases of hypertension. Catecholamine-secreting tumors that arise from chromaffin cells of the adrenalAdrenal Gland Steroids. Read more ... » medulla and the sympathetic ganglia are referred to as “pheochromocytomas” and “extra-adrenalAdrenal Gland Steroids. Read more ... » catecholamine-secreting paragangliomas” (“extra-adrenalAdrenal Gland Steroids. Read more ... » pheochromocytomas”), respectively Pheochromocytomas are a group of cells which produce and secrete catecholamines. They are derived from adrenal medulla or chromaffin cells of the sympathetic ganglia. There is sinus tachycardia, bradycardiaSINUS Bradycardia Bradyarrhythmia Symptoms Causes Diagnosis with Treatment. Read more ... », supraventricu­lar arrhythmiasCOMPLICATIONS OF Acute Myocardial Infarction (AMI) AND TREATMENT -2. Read more ... », ventricular premature contractions, angina and acuteMetabolic,Insulin Resistance Syndrome X Causes Symptoms. Read more ... » myocardial infarction.

CLINICAL PRESENTATION —

  • The most common feature of pheochromocytoma is hypertension and paroxysmal hypertension which may be very disturbing.
  • It is a correctable cause of hypertension.
  • Untreated pheochromocytomas may be fatal. Pheochromocytomas are situated in the adrenalAdrenal Gland Steroids. Read more ... » medulla or in abdomen near the celiac, superior me­senteric and inferior mesenteric ganglia (sympathetic ganglia).
  • It occurs at all ages but common in young age. It is more common in females.
  • There is labile hypertension, seizure disorders, anxiety attacks. Sometimes it is detected during surgery or after trauma.
  • There is headache, excessive sweating and palpita­tions.
  • There may be pain in chest and abdomen, with nau­sea and vomiting.
  • There may be sudden flushing.
  • Pheochromocytoma is usually suggested by the history in a symptomatic patient, discovery of an incidental adrenalAdrenal Gland Steroids. Read more ... » mass, or the family history in a patient with familial disease.

Diagnosis

When to suspect pheochromocytoma —

  • Hyperadrenergic spells (eg, self-limited episodes of nonexertional palpitations, diaphoresis, headache, tremor, or pallor)
  • Resistant hypertension
  • A familial syndrome that predisposes to catecholamine-secreting tumors (eg, MEN2, NF1, VHL)
  • A family history of pheochromocytoma
  • An incidentally discovered adrenalAdrenal Gland Steroids. Read more ... » mass Hypertension and diabetes Pressor response during anesthesia, surgery, or angiography Onset of hypertension at a young age (eg, <20 years)
  • Idiopathic dilated cardiomyopathy
  • A history of gastric stromal tumor or pulmonary chondromas (Carney triad)

Treatment Pheochromocytoma

  • Phenoxybenzamine, an alpha receptor blocker, in dose of 10 mg 12 hourly, increasing 10 mg every few days, till blood pressure is controlled and paroxysms disappear.
  • For tachycardia, after alpha blockers, beta blockersMANAGEMENT of Acute Myocardial Infarction (AMI) -1. Read more ... » may be given in low dose.
  • The tumors may be localized by eT, MRI and re­moved surgically.
  • Once a pheochromocytoma is diagnosed, all patients should be operated on after appropriate medical preparation.
  • Extirpating a pheochromocytoma is a high-risk surgical procedure, and an experienced surgeon/anesthesiologist team is required.