Pheochromocytoma natural history, complications and prognosis

Pheochromocytoma is an adrenaline secreting tumor, that usually develops in the fifth decade of life. Symptoms start with tachycardia, hypertension, headache and sweating. Massive release of catecholamines may cause hyperglycemia, malignant hypertension and metastasis. The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 50%.

• The symptoms of pheochromocytoma usually develop in the fifth decade of life.
• Common symptoms are with tachycardia, hypertension, headache, and sweating.
• If left untreated, hyperglycemia and hypertensive emergency. It may lead to heart failure andcerebrovascular strokes.
• If malignant, It can metastasize to lymph nodes, bones, lungs, and liver. ^[1]

Common complications of pheochromocytoma include:

• Damage to cardiac myocytes due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on cardiac myocytes.^[2]
• Hyperglycemia due to opposition of insulin effect by high doses of adrenaline secreted by the tumor.
• Malignant hypertension that may cause cerebrovascular accidents such as:
  • Intracranial hemorrhage
  • Acute coronary syndrome
  • Aortic dissection
  • Heart failure
• Metastasis to:
  • Lymph nodes
  • Bones
  • Lungs
  • Liver

• The prognosis of pheochromocytoma is generally good with treatment. The 5-year survival rate in patients with metastatic pheochromocytoma is approximately 50%.^[3]
• Prognosis and survival rate varies with the location of the primary tumor, sites of metastases, Tumor burden, and rate of progression.
• Metastasis to the brain and liver has a worse prognosis than other metastases.

Post-surgical prognosis

• Factors associated with a favourable prognosis include:
  • Small tumor size
  • Short duration of surgery
  • Systolic blood pressure < 160 mmHg
  • Low levels of urinary catecholamines.^[4]
• Approximately 10% recur after being resected.
• Recurrence is more common in patients with familial pheochromocytoma and extra-adrenal tumors.^[5]