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pheochromocytoma-pathogenesis-and-clinical-findings

Pheochromocytoma: Pathogenesis and clinical findings
Author: Jaye Platnich Huneza Nadeem Reviewers: Mark Elliott Alexander Arnold Ran (Marissa) Zhang Hanan Bassyouni* * MD at time of publication
↑ Blood pressure results in the activation of neural pain receptors
Sustained or paroxysmal 2o hypertension (↑ blood pressure)
Pallor
Tachycardia (↑ heart rate), palpitations
Diaphoresis (excessive sweating)
Hyperglycemia Weight loss, fatigue
  Familial Disorders (10%)
E.g., Multiple Endocrine Neoplasm 2 Syndrome (MEN2) types A and B, Neurofibromatosis 1 (NF-1), Von Hippel Lindau Syndrome (VHL), familial pheochromocytoma
Dysfunction of various tumor suppressor and/or oncogene proteins
Uncontrolled proliferation of the chromaffin cells in the medulla of the adrenal gland(s)
Adenoma formation (10% bilateral)
Over-production of epinephrine and
norepinephrine from the adrenal adenoma(s) or extra- adrenal tumour (10%)
Detectable metanephrines (epinephrine/norepinephrine breakdown products) in both plasma and urine
Sporadic DNA mutations arising from
DNA damage from exposure to mutagens, malignancy (10%), or during DNA replication
Detectable mutations on the Von Hippel Linda (VHL), (Rearranged During Transcription) RET, (Succinate Dehydrogenase) SDH, and/or other tumour suppressor or oncogenes
Visible adrenal mass on CT scan Heart attack, stroke, or death (Note: These
tumors can be fatal therefore screening is essential in patients with adrenal masses or 2o hypertension)
Episodic hyper-activity of the sympathetic nervous system
Hyper-stimulation of G protein-coupled receptors involved in catabolic metabolic processes
Headaches
         ↑ Vasoconstriction of peripheral blood vessels
Hyper-stimulation of adrenergic receptors on cardiac myocytes
↑ Secretion from the eccrine sweat glands
Panic, tremor, anxiety
↑ Ability to mobilize glucose into the bloodstream through enhanced lipolysis, glycogenolysis and gluconeogenesis
                     Legend:
 Pathophysiology
 Mechanism
Sign/Symptom/Lab Finding
 Complications
Published February 20, 2014, updated June 27, 2022 on www.thecalgaryguide.com