pheochromocytoma
DESCRIPTION
endocrinology - increase nor adrenalinTRANSCRIPT
PHEOCHROMOCYTOMA
Hamad Emad H. Dhuhayr
CONTENTS
• SOEPEL
• PHEOCHROMOCYTOMA
• REFFERENCES
SOEPEL
S A 35-year-old husband and father of three children, has been experiencing headaches
and palpitations of increasing frequency and severity over the past six months. In
addition, he has had periods of intense anxiety and panic attacks.
• O taking history and physical examination.
• E Anxiety disorders, hyperthyroidism and pheonchomocytoma
• P Echo and ecg
• E medication.
• L pheonchomocytoma
PHEONCHOMOCYTOMA
DEFINITION
• Catecholamine-secreting tumors that arise from chromaffin cells of theAdrenal
medulla and the sympathetic ganglia are referred to as pheochromocytomas
And extra-adrenal catecholamine-secreting paragangliomas, respectively.
• Because the tumors have similar clinical presentations and are treated With
similar approaches, many clinicians use the term pheochromocytoma to Refer to
both entities.
• However, the distinction between pheochromocytoma And paraganglioma is an
important one because there are differences in the Risk for associated
neoplasms, risk for malignant transformation, and type of Genetic testing that
should be considered.
• Rare but treatable.
PATHOLOGY
• Tumor of chromaffin tissue.
• 90% from adrenal medulla & 10% from other chromaffin tissue from the carotid
down to Urinary bladder.
• 90% benign and 10% malignant
• 90% is unilateral
• 10% multiple tumors.
CLINICAL PICTURE
COMPLICATION
1. CNS: stoke secondary to hypertension
2. CVS: ischaemic heart disease (coronary spasm + HTN),
cardiomyopathy (catecholamines Induced myo-
necrossis+hypertension)
3. DM: due to insulin antagonism.
4. sudden death due to arrhythmia
ASSOCIATIONS
• Neurofibromatosis
• MEN-ii
• Hypertrophic cardiomyopathy
• Von-hipple lindeau “VHL” syndrome: CNS and retinal
hemangioblstomas, renal and pancreatic cysts, and
hypernephroma.
• Sturge weber syndrome: CNS a-v malformation, and cutanouse
angioma of the face.
INVESTIGATIONS
1- hormone function:
- ECG, ECHO, and blood glucose level.
- suppressor test: clonidine injection iv 5mg → ↓ of BP by 53/25 mmhg for 15
minutes Due to blockade of alpha-receptors in other forms of hypertension but
not in Pheochromocytoma.
2- hormone level:
- serum catecholamines level is elevated (n=2-5mg/L)
- neurophysin serum level is elevated (secreted in equimolar concentration with
epinephrine)
3- hormone end product: 24hrs urinary vallinyl mandelic acid (VMA) is elevated
(the urine
Should be collected in acidic medium and refrigerated). The result of the test is
falsely high
In patients taking alpha-methyl dopa, banana or pine apple (n=2-6 mg/24 hrs).
4- of cause:
- CT, MRI or sonography of abdomen
- iodine 131 -metaiodobenzyl guanidine (MIBG) scanning, particularly in extra-
abdominal
Tumors.
- IVP may show indentation of the upper pole of kidney
TREATMENT
• 1- hormone action: α-blocker (phenoxybenzamine 20-
40 mg/day) followed by β-blocker (propranolol 120-240
mg/day) or combined α and β blocker (labetalol)
• 2- surgical removal of the tumor.
FOLLOW UP
• The 24-hour urinary excretion of fractionated catecholamines and
metanephrinesOr plasma fractionated metanephrines should be checked
annually For life.
• Annual biochemical testing assesses for metastatic disease, tumor Recurrence
in the adrenal bed, or delayed appearance of multiple primaryTumors.
• Follow-up CT or MRI is not needed unless the metanephrine or catecholamine
Levels become elevated or the original tumor was associated with Minimal or no
catecholamine or metanephrine excess.
REFFERENCES
• KUMAR
• CEICEL
• DAVIDSONE
• WEBSITE