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Pheochromocytoma – Quick revision card

Neuroendocrine tumor Pheochromocytoma( PCC ) -are neuroendocrine tumors of the adrenal medulla ( chromaffin cell )

 

Paraganglion -from the sympathetic and parasympathetic ganglion (PGL )

 

incidence ranges between 2 and 8 per million picked up incidentally and of them 80-85 % are PCCs and 15 % to 20 % are PGLs in out patient clinics these occur in 0.1 % and 0.6 % of patients with hypertension they can appear at any age but more common in 3rd to 5th decade hereditary disease is more likely to manifest in younger patients

 

PCC’s mostly arise from the adrenal medulla whereas PGLs tend
 to arise from sympathetic or parasympathetic ganglia outside the of the adrenal medulla for e.g retroperitoneum , pelvis , thorax , head and neck

 

Up to 70 % of PPGLs are caused by germline or somatic variants in one of the the susceptibility gene
 ( ie a hereditary tumor ) More than 20 mutations are known

 

Symptoms can manifest due to excessive production of catecholamines 
( epinephrine , norepinephrine and dopamine )

 

Hypertensive crisis palpitations ,diaphoresis , headache ( classic triad ) + hypertension due to massive release of catecholaminergic hormones from the tumor ( and resultant haemodynamic and metabolic actions ) episodes may last from few minutes to a few hrs can be precipitated by events as stress , surgery , anesthetics , intense physical effort , palpation of lumbar region

 

paroxysms of hypertension resistant hypertension non-specific symptoms (nausea , pallor , constipation , flushing , wight loss , fatigue , anxiety , orthostatic hypotension , fever , hyperglycemia )

 

Pressure effect or metastasis end-organ damage due to long standing hypertension ( presentation with complications )

 

A difficult diagnosis to establish as numerous clinical conditions can present with similar signs and symptoms , hence pheos are often referred to as Great Mimic or The Great Masquerader

 

Often detected as an incidental ” asymptomatic ” adrenal mass and on further evaluation the classical symptoms of Pheo / PGL can be elicited in retrospect

 

measurement of urinary and plasma catecholamines urinary fractionated and plasma free metanephrines urinary vanillylmandelic acid ( VMA ) imaging studies as CT

 

if left untreated high CV mortality detection may help earlier diagnosis in other family members some PPGL’s have malignant potential.

REFERENCES

  1. Pheochromocytoma and Paraganglioma Diagnosis, Genetics, and Treatment Colleen M. Kiernan, MD, MPH*, Carmen C. Solórzano, MD1  Surg Oncol Clin N Am 25 (2016) 119–138 *Pheochromocytoma and Paraganglioma (cbc.org.br)
  2. Pheochromocytoma Frederick-Anthony Farrugia1 , Anestis Charalampopoulos2 1 General Surgeon, Private practice, Athens, Greece; 2 Third Department of Surgery, Attikon University Hospital,
    Medical School of Athens, Athens, Greece ENDOCRINE REGULATIONS, Vol. 53, No. 3, 191–212, 2019
  3. Manea, Maria; Marcu, Dragos R.; Bratu, Ovidiu G.; Stanescu, Ana M.; Pantea Stoian, Anca; Gaman, Mihnea A.; and Diaconu,
    Camelia C. (2019) “Pheochromocytoma – clinical manifestations, diagnosis and current perioperative management,” Journal of Mind
    and Medical Sciences: Vol. 6 : Iss. 2 , Article 10.
    DOI: 10.22543/7674.62.P243247
  4. Aygun, Nurcihan, and Mehmet Uludag. “Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical Findings.” Sisli Etfal Hastanesi tip bulteni vol. 54,2 159-168. 3 Jun. 2020, doi:10.14744/SEMB.2020.18794 Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical Findings (nih.gov)

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