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  • • Episodic headache, excessive sweating, palpitations, and visual blurring

    • Hypertension, frequently sustained, with or without paroxysms

    • Postural tachycardia and hypotension

    • Elevated urinary catecholamines or their metabolites, hypermetabolism, hyperglycemia

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Epidemiology

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  • • Less than < 0.1% of patients with hypertension

    • 5% of tumors discovered incidentally on CT scan

    • Most occur sporadically

    • Associated with familial syndromes, such as:

    • -Multiple endocrine neoplasia type 2A (MEN 2A)

      -MEN 2B

      -Recklinghausen disease

      -von Hippel-Lindau disease

    • Pheochromocytomas are present in 40% of patients with MEN 2

    • 90% of patients with pheochromocytoma are hypertensive

    • Rule of 10s:

    • - 10% malignant

      -10% familial

      -10% bilateral

      -10% multiple tumors

      -10% extra-adrenal

    • Hypertension less common in children

    • In children, 50% of patients have multiple or extra-adrenal tumors

    • Extra-adrenal pheochromocytomas:

    • -Abdomen (75%)

      -Bladder (10%)

      -Chest (10%)

      -Pelvis (2%)

      -Head and neck (3%)

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Symptoms and Signs

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  • • Clinical findings are variable

    • Episodic or sustained hypertension

    • Triad of palpitation, headache, and diaphoresis

    • Anxiety, tremors

    • Weight loss

    • Dizziness, nausea, and vomiting

    • Abdominal discomfort, constipation, diarrhea

    • Visual blurring

    • Tachycardia, postural hypotension

    • Hypertensive retinopathy

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Laboratory Findings

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  • • Hyperglycemia

    • Elevated plasma metanephrines

    • Elevated 24-hour urine metanephrines and free catecholamines

    • Elevated urinary vanillylmandelic acid (VMA)

    • Elevated plasma catecholamines

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Imaging Findings

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  • • Adrenal mass seen on CT or MRI

    • Characteristic bright appearance on T2-weighted MRI

    • Asymmetric uptake on MIBG scan. Particularly useful for extra-adrenal, multiple, or malignant pheochromocytomas. Not useful for sporadic biochemical syndrome with unilateral mass

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  • • Avoid arteriography or fine-needle aspiration as they can precipitate a hypertensive crisis

    • Early recognition during pregnancy is key because if left untreated, half of fetuses and nearly half of the mothers will die

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Rule Out

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  • • Other causes of hypertension

    • Hyperthyroidism

    • Anxiety disorder

    • Carcinoid syndrome

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  • • History and physical exam

    • -Suspect pheochromocytoma based on symptoms

    • CT, MRI, or other scans

    • Plasma and urine studies (metanephrines, catecholamines, VMA)

    • Begin treatment with α-blockers

    • Possible MIBG scan

    • Operative excision of tumor

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When to Admit

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  • • Hypertensive crisis (can develop multisystem organ failure, mimicking severe sepsis)

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  • • α-Adrenergic blocking agents should be started as soon as the biochemical diagnosis is established to restore blood volume, to prevent a severe crisis, and to allow recovery from the cardiomyopathy

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Surgery

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Indications

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  • • All pheochromocytomas should be excised

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Contraindications

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  • • Metastatic disease

    • Inadequate medical preparation ( blockade)

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Medications

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  • • -Adrenergic blocking agents, such as phenoxybenzamine

    • Other agents include metyrosine, prazosin, and calcium channel blockers

    • β-Adrenergic blocking agents can be used only after full blockade has been achieved

    • Avoid opioids ...

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