This document discusses pheochromocytoma, a rare catecholamine-secreting tumor that arises from chromaffin cells of the adrenal medulla. It notes that pheochromocytomas have an annual incidence of approximately 0.8 per 100,000 people. Symptoms can include paroxysmal or primary hypertension, headaches, sweating, and tachycardia. Diagnosis involves biochemical testing for catecholamines and metanephrines in urine or plasma. Treatment consists of preoperative medical management with alpha- and beta-blockers to control blood pressure, followed by surgical adrenalectomy.

1. phaeochromocytoma
Dr Abdirahman Ibrahim said (MBBS,MD )

2. • Catecholamine-secreting tumors that arise from chromaffin cells of
the adrenal medulla are referred to as "pheochromocytomas“.
• It is estimated that the annual incidence of pheochromocytoma is
approximately 0.8 per 100,000 person-years
• The prevalence of phaeochromocytoma in patients with hypertension
is estimated to be 0.1 -0.6% in black South Africans

3. • Most catecholamine-secreting tumors are sporadic. However,
approximately 40 percent of patients have the disease as part of a
familial disorder.

4. Clinical manifestations

5. • Approximately one-half have paroxysmal hypertension; most of the
rest have either primary hypertension (formerly called "essential"
hypertension) or normal blood pressure.

6. • Less common symptoms and signs
• orthostatic hypotension
• Cardiomyopathy Rarely, pheochromocytoma is associated with
cardiomyopathy attributed to catecholamine excess that is similar to
stress-induced (takotsubo) cardiomyopathy .
• Paroxysmal elevations in blood pressure – Patients with symptoms
related to paroxysmal elevations in blood pressure (hypertension,
tachycardia, or arrhythmia)
• Asymptomatic patients

7. Indications for testing
• The classic triad of headache, sweating, and tachycardia, whether or not
they have hypertension.
• Hyperadrenergic spells (eg, self-limited episodes of nonexertional
palpitations, diaphoresis, headache, tremor, or pallor).
• Onset of hypertension at a young age (eg, <20 years), resistant
hypertension, or hypertension with new-onset or atypical diabetes mellitus
(eg, new onset of apparent type 2 diabetes in a slender person).

8. • A familial syndrome that predisposes to catecholamine-secreting
tumors (eg, multiple endocrine neoplasia type 2 [MEN2],
neurofibromatosis type 1 [NF1], or von Hippel-Lindau [VHL]).
• A family history of pheochromocytoma.
• adrenal incidentaloma
• Idiopathic dilated cardiomyopathy.
• a history of gastric stromal tumor or pulmonary chondromas (Carney
triad)

9. Low risk for pheochromocytoma
• 24-hour urinary fractionated catecholamines and metanephrines
should be the first test in patients with a somewhat lower index of
suspicion for pheochromocytoma. This includes patients with:

10. High risk for pheochromocytoma
• High risk for pheochromocytoma — The index of suspicion for a catecholamine-
secreting tumor should be high for the following scenarios:
• A family history of pheochromocytoma.
• A genetic syndrome that predisposes to pheochromocytoma (eg, MEN2).
• A past history of resected pheochromocytoma.
• An incidentally

11. High risk for pheochromocytoma
•Plasma fractionated metanephrines

12. ADDITIONAL EVALUATION AFTER
BIOCHEMICAL DIAGNOSIS
•CT and MRI
• Increased attenuation on nonenhanced CT (most are >20
Hounsfield units [HU])
• Increased mass vascularity
• High signal intensity on T2-weighted MRI
Cystic and hemorrhagic changes
• Variable size and may be bilateral

13. CON’T
• PET
• Genetic testing

14. MEDICAL PREPARATION FOR SURGERY
• surgical mortality rates are increased due to lethal hypertensive
crises, malignant arrhythmias, and multiorgan failure.
• Combined alpha and beta-adrenergic blockade

15. • Phenoxybenzamine
• The initial dose is 10 mg once or twice daily, and the dose is increased
by 10 to 20 mg in divided doses every two to three days as needed to
control blood pressure and spells
• The final dose of phenoxybenzamine is typically between 20 and 100
mg daily.

16. • With their more favorable side-effect profiles and lower financial cost,
selective alpha-1-adrenergic blocking agents (eg, prazosin, terazosin,
or doxazosin) are utilized in many centers or are preferred to
phenoxybenzamine when long-term pharmacologic treatment is
indicated (eg, for metastatic pheochromocytoma).

17. Blood pressure monitoring
• Blood pressure monitoring — Blood pressure should be monitored twice
daily in the outpatient setting with the patient in the seated and standing
positions.
• Target blood pressure is low-normal blood pressure for age (eg, less than
120/80 mmHg seated), with systolic blood pressure greater than 90 mmHg
(standing); both targets should be modified on the basis of the patient's
age and comorbid disease.

18. High sodium diet
• On the second or third day of alpha-adrenergic blockade, patients are
encouraged to start a diet high in sodium content (>5000 mg daily)
because of the catecholamine-induced volume contraction and the
orthostasis associated with alpha-adrenergic blockade.

20. BETA BLO CKERS
• a patient may be given 10 mg of propranolol orally every six hours
on the first day of beta-adrenergic blockade. Another option is to
start with low-dose metoprolol (eg, 12.5 mg twice daily).
• Calcium channel blockers

21. ADRENALECTOMY

22. Other complication
• Acute hypertensive crises sodium nitroprusside, phentolamine, or
nicardipine.
• Cardiac arrhythmias :managed with lidocaine (50 to 100 mg intravenously)
or esmolol (50 to 200 mcg per kg per minute intravenously).
• Postoperative hypoglycemia has been reported in patients undergoing
adrenalectomy for pheochromocytoma in 4 to 43 percent of patients based
upon serum glucose cutoffs of <55 mg/dL

23. Adrenal insufficiency

24. • Adrenal insufficiency is a condition in which the adrenal glands do not
produce adequate amounts of steroid hormones

25. Causes of primary adrenal insufficiency
• Autoimmune
• Malignancy
• Metastatic
• Lymphoma
• infiltration
• Infection
• Tuberculosis
• Opportunistic infections
• AIDS

26. Con’t
• Vascular haemorrhage
• Waterhouse–Friderichsen syndrome
• Congenital adrenal hyperplasia
• Triple A syndrome (Achalasia, Addisonianism, Alacrimia)
• Iatrogenic

27. Clinical manifestation
• Patients with chronic primary adrenal insufficiency may have
symptoms and signs of glucocorticoid, mineralocorticoid, and, in
women, androgen deficiency.

28. • Features of both primary and secondary adrenal insufficiency
• Fatigue
• Weight loss
• Gastrointestinal complaints
• Amenorrhea
• Musculoskeletal
• Psychiatric

29. Features of primary adrenal insufficiency
• Hyperpigmentation
• Salt craving
• Hypotenstion

30. Laboratory findings
• Electrolyte abnormalities :Hyponatremia, Hyperkalemia
• Hypoglycemia
• Hematologic findings

31. • Demonstrating inappropriately low cortisol secretion
• Determining whether the cortisol deficiency is dependent on or independent of
corticotropin (ACTH) deficiency and evaluating mineralocorticoid secretion in patients
without ACTH deficiency
• Seeking a treatable cause of the primary disorder (eg, infiltrative process involving the
adrenal glands or a pituitary adenoma compromising normal pituitary function)

32. • Stage 1: High plasma renin activity and normal or low serum
aldosterone
• Stage 2: Impaired serum cortisol response to ACTH stimulation
• Stage 3: Increased morning plasma ACTH with normal serum cortisol
• Stage 4: Low morning serum cortisol and overt clinical adrenal
insufficiency

33. Con’t
• cortisol concentration
• Serum cortisol concentration
• Morning serum cortisol concentration
• Morning salivary cortisol concentration
• Urinary cortisol measurements
• ACTH stimulation tests

34. ESTABLISHING THE LEVEL OF DEFECT
• Basal plasma ACTH, renin, and aldosterone concentration
• Response to prolonged ACTH stimulation —
• Insulin-induced hypoglycemia test
• Corticotropin-releasing hormone test

35. DETERMINING THE ETIOLOGY
• Pituitary computed tomography (CT) or magnetic resonance imaging
(MRI)
• Measurement of antiphospholipid antibodies
• Chest radiograph, urine culture for Mycobacterium tuberculosis, and
tuberculin skin testing

36. Glucocorticoid replacement
• Hydrocortisone 15 to 25 mg orally in two or three divided doses (largest
dose in morning upon awakening; typically 10 mg upon arising in morning,
5 mg early afternoon, 2.5 mg late afternoon)
• Prednisone 5 mg (range: 2.5 to 7.5 mg) orally at bedtime
• Dexamethasone 0.75 mg (range: 0.25 to 0.75 mg) orally at bedtime

37. • Mineralocorticoid replacement:
• Fludrocortisone 0.1 mg (range: 0.05 to 0.2 mg) orally.
• Androgen replacement
• Dehydroepiandrosterone (DHEA) initially 25 to 50 mg orally
• Patient education
• Treatment of minor febrile illness or stress (known as the 3 x 3 rule).

38. The end