Pheochromocytoma

Supervised by: dr. Intessar sultan

Presented by:
•Neama shabib 1621161
•Dalal alnajjar 1621149
•Jana babaqi 1621162
Learning objectives
At the end of the lecture the student will be able to:
• Define pheochromocytoma- paraganglioma
• Identify underlying causes of pheochromocytoma
• Identify the associated familial syndromes with pheochromocytoma
• Recognize the different clinical presentations of pheochromocytoma
• Identify how to diagnose pheochromocytoma
• Identify how to manage pheochromocytoma
Cromaffine cells (pheochromocyte) : orginate from the neural crust (embryo)
Neuroendocrine cell > neurohormones ( chemicals produced from neuron cells released on the blood)
- adrenaline
- noradrenaline
Migrates to areas adjacent to the sympathetic ganglia:
adrenal medulla : phenochromocytoma
Others : paraganglioma or extra-adrenal
 Definition
,Pheochromocytoma is a rare benign neuroendocrine tumor •
Arise from •
chromaffin cells of the adrenal medulla
sympathetic ganglia (extra-adrenal pheochromocytomas ‘’paraganglioma’’)
.Active tumor : Secretion of excess catecholamines •
Prevalence among hypertensive patients is only 0.2% •
of cases are discovered incidentally during abdominal CT or MRI 60% •
serious cardiovascular complications •
:Tumor characteristics Rule f 10
• 10% multiple.
• 10% extraadrenal (catecholamine-secreting paragangliomas.
• 10% malignant:
• known only by local invasion or metastases not by Histopathology
• So long-term follow-up is indicated in all patients to exclude malignancy
• Patients with the succinate dehydrogenase (SDH) subunit B mutations are
more likely to develop metastatic disease
Symptoms (50% of cases)
• the clinical presentation may be variable
• the classic triad: episodic headache, sweating, and tachycardia in association with
hypertension.
• Paroxysmal elevations in blood pressure
• Orthostatic hypotension: low plasma volume – secretion of epinephrine
• Cardiomyopathy
• insulin resistance- diabetes
• Rare: pheochromocytoma multisystem crisis.
• hypertension or hypotension, hyperthermia (temperature >40°C), mental status
changes, and organ dysfunction
Familial cases
of pheochromocytoma cases 40% •
Bilateral adrenal tumors or multiple paragangliomas •
Autosomal dominant •
Succinate dehydrogenase subunit gene variants SDH •
Multiple endocrine neoplasia type 2 (MEN2): RET oncogen -1
MEN2A: 1ry hyperparathyroidism, medullary thyroid carcinoma, pheochromocytoma •
MEN2B: medullary thyroid carcinoma, pheochromocytoma, mucocutanous neuroma •
von Hippel-Lindau (VHL-II) disease (retinal angiomas,rena cell carcinoma, -2
hemangioblastoma) 3- Neurofibromatosis NF 1: neurofibroma, café au lait spots,
axillary freckling
Neurofibromatosis
Differential diagnosis
• Anxiety
• Thyrotoxicosis
• Angina
• Hypoglycaemia
Indication for Evaluation
• Suspected cases (low and high index)
• Adrenal incidentalome
• paroxysmal elevations in blood pressure ( precipitating factors)
• diagnostic procedures as colonoscopy
• induction of anaesthesia
• Surgery
• consumption of food high in tyramine
• with certain drugs (beta blockers, tricyclic antidepressants, corticosteroids,
metoclopramide or MAOIs
Initial biochemical tests
1- Low index of suspicion: 24-hour urinary catecholamines and metanephrines.
• Resistant hypertension
• Hyperadrenergic spells
2- High index of suspicion: plasma metanephrines
• Family history of pheochromocytoma
• Genetic syndrome as MEN2
• Past history of pheochromocytoma
• Imaging criteria of adrenal incidentalome
 Imaging
Imaging studies should be performed only after biochemical studies have confirmed
the diagnosis of pheochromocytoma.
• CT/MRI can detect tumors but non specific due to high prevalence of adrenal
incidentalomas
•If CT/MRI negative with high suspicions: MIBG scan
•If suspect metastasis: PET scan
MRI
MIBG PET
Genetic testing
if a patient has one or more of the following •
Paraganglioma .1
Bilateral adrenal pheochromocytoma .2
Unilateral adrenal pheochromocytoma and a family history of .3
pheochromocytoma/paraganglioma
Unilateral adrenal pheochromocytoma onset at a young age less than 45 .4
Other clinical findings suggestive of one of the syndromic disorders .5
Surgery for adrenal pheochromocytomas
• Surgery is indicated for patients with biochemical documentation
• Laparoscopic adrenalectomy is better than open surgery
• Complete bilateral adrenalectomy in familial cases with diffuse disease to
avoid recurrence
Perioperative preparation
• Start with alpha-adrenergic blockade: phenoxybenzamine is the first-line
• Add low dose beta blocker cautiously after adequate alpha blockade
• Increase dose to control tachycardia (goal 60 - 80 BPM).
• NEVER start with beta-adrenergic blocker.
• Intraoperative:
• manage with intravenous saline to hydrate patient as after removal of the
tumour severe hypotension and shock may develop
REFERENCE