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