Diagnosis

Pheochromocytoma

A tumor arising from adrenomedullary chromaffin cells most commonly in the adrenal medulla (> 90%) that commonly produces catecholamines: epinephrine, norepinephrine, and/or dopamine [hz1][nsf]

Etiology

Cause [nth]

Pathophysiology [nth]

  1. Catecholamines (dopamine --> norepinephrine --> epinephrine) are a group of hormones and neurotransmitters essential for maintaining homeostasis and regulating the body's response to stress

  2. Intermittently or continuously excessive secretion

  3. Episodic hypertension, headaches, diaphoresis, palpitations, sweating, and flushing

  4. Hypertensive crisis: cardiovascular shock, stroke, myocardial infarction, or multiorgan failure

  5. Asymptomatic (incidentaloma): 40%

Complications [nth]

Epidemiology

Incidence per 100.000 [pmc][a2o][23r][ivp][tcg][hzi][rsv][hz1]

Epidemiology chart for Incidence

Symptoms & findings

Symptoms

Anxiety, Cafe au lait spot, Constipation, Diaphoresis, Dizziness, Headache, Heat intolerance, Hypertension, Ileus, Nausea, Neurofibroma, Pallor, Palpitations, Polydipsia, Polyuria, Tachycardia, Tremor, Vomiting, Weakness

Clinical findings

Elevated Hematocrit, Elevated Hemoglobin, Elevated Metanephrines, Elevated Normetanephrines, Elevated Vanillylmandelic Acid, Polycythemia

Anamneses

None listed.

Localized findings

Pain
Radiates
CaputAbdomen
Onset
Acute (minutes)Subacute (hours)
Pattern
IntermittentRecurring
Severity
Mild (1-3)Moderate (4-7)Severe (8-10)
Rash
Radiates
CaputCollumThoraxAbdomenGenitalArmBackLower body
Distribution
GeneralizedMultipleSymmetric
Lesion type
Nodule
Rash
Radiates
CaputCollumThoraxAbdomenGenitalArmBackLower body
Distribution
MultipleSingle
Lesion type
Macule
Color
Brown

Approach

Treatment

  1. Preoperative α-blockade: henoxybenzamine, prazosin, doxazosin, or urapidil [hz1][ket]

  2. β-blockers after sufficient α-blockage due to vasoconstriction: propranolol or atenolol

  3. Laparoscopic (adrenal sparing) resection

  4. Embolization in Pheochromocytoma

  5. Malignant pheochromocytoma: 5-year survival rates of 50% [ket]

    • Radical adrenalectomy

    • Myeloablative chemotherapy?

Differential diagnoses

Acute intermittent porphyria, Alcohol withdrawal, Angina pectoris, Anxiety, Arrhythmia, Autonomic neuropathy, Carcinoid syndrome, Carcinoid tumor, Cardiomyopathy, Cushing syndrome, Drug side effects, Epilepsy, Essential hypertension, Heart failure, Hyperaldosteronism, Hyperthyroidism, Hyperventilation, Hypoglycemia, Insulinoma, Lead toxicity, Mastocytosis, Meningioma, Menopause, Migraine, Orthostatic hypotension, Paroxysmal supraventricular tachycardia, POEMS syndrome, Polyneuropathy, Preeclampsia, Pulmonary edema, Renal artery stenosis, Stroke, Subarachnoid hemorrhage, Thyroid cancer

References

[1] Reisch N, Peczkowska M, Januszewicz A, Neumann HP. Pheochromocytoma: presentation, diagnosis and treatment. J Hypertens. 2006 Dec;24(12):2331-9.

[2] Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99(6):1915-42.

[3] Gupta PK, Marwaha B. Pheochromocytoma. [Updated 2024 Nov 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK589700/

[4] Al Subhi AR, Boyle V, Elston MS. Systematic Review: Incidence of Pheochromocytoma and Paraganglioma Over 70 Years. J Endocr Soc. 2022 Jul 3;6(9):bvac105.

[5] Kim JH, Moon H, Noh J, Lee J, Kim SG. Epidemiology and Prognosis of Pheochromocytoma/Paraganglioma in Korea: A Nationwide Study Based on the National Health Insurance Service. Endocrinol Metab (Seoul). 2020 Mar;35(1):157-164.

[6] Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, Kerstens MN. Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review. Eur J Intern Med. 2018 May;51:68-73.

[7] Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT. Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979. Mayo Clin Proc. 1983 Dec;58(12):802-4.

[8] Yeo H, Roman S. Pheochromocytoma and functional paraganglioma. Curr Opin Oncol. 2005 Jan;17(1):13-8.

[9] Stenström G, Svärdsudd K. Pheochromocytoma in Sweden 1958-1981. An analysis of the National Cancer Registry Data. Acta Med Scand. 1986;220(3):225-32.

[10] Guerrero MA, Schreinemakers JM, Vriens MR, Suh I, Hwang J, Shen WT, Gosnell J, Clark OH, Duh QY. Clinical spectrum of pheochromocytoma. J Am Coll Surg. 2009 Dec;209(6):727-32.

[11] Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005 Aug 20-26;366(9486):665-75.

[12] http://emedicine.medscape.com/article/124059 (2014-01-02); [Medscape]

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