Phaeochromocytoma

1. Pheochromocytoma Amina Al- Qaysi

2. Adrenal gland Outer cortex: Zona Glomerulosa , Zona Fasciculata , Zona Reticularis Inner medulla: Chatecholamines

3. Case A 29 years old man presents to the ER complaining of crushing headache & palpitations . He gives a history of previous similar episodes . Physical examination reveals a pulse of 140 BPM, BP of 200/110 mmHg.

4. Pheochromocytoma Neuroendocrine tumor, Adrenal medulla Secretes chatecholamines The 10% Tumor … Gross : Circumscribed lesion, hemorrhagic & necrotic areas Microscopic: Nests of polygonal chromaffin cells

5. Epidemiology Occur at any age, peak at 3 rd -5 th decade M=F Sporadic, Hereditary Associations : Von- Hipple Lindau Disease, NF 1, MEN 2, Tuberous sclerosis, Sturge-weber $

6. Clinical Presentation General features: Sweating, Flushing, heat intolerance, pallor, Weakness, pyrexia. Neurological: Headache, Tremors, visual disturbances, Anxiety, panic attacks, seizures. Cardiovascular: Hypertension, Palpitations, chest tightness, dyspnea , faints.

7. Clinical Presentation Cont’d Gastrointestinal: Abdominal pain, Nausea, Weight loss, constipation. Asymptomatic.

8. Clinical Presentation Cont’d Precipitating events: Exercise General anesthesia induction Labour Stress IV contrast agents TCA

9. Investigations Demonstrate excess chatecholamines Tumor localization

10. Investigations Cont’d 24-hours urine collection Plasma Metanephrine levels

11. Investigations Cont’d MRI (Preferred study): Hyper-intense on T2, No contrast required CT scan ?? SPECT

12. SPECT I-MIBG (Meta- iodobenzylguanidine ) Single Photon Emission Computerized Tomography Identify 90% of primary tumors Detects multiple extra-adrenal tumors & metastases

13. Management Surgical resection of the mass is the treatment of choice Medical treatment : Pre-operatively, for management of hypertensive crises. Alpha blockers ( Phenoxybenzamine ) Beta blockers: Propranolol . If arrhythmia or tachycardia occur.

14. Medical Management Phenoxybenzamine : Start as 10 mg orally twice aday Increase to 20-40 mg orally twice aday (until the desired effect or the adverse effects occur) AE : Postural hypotension, tachycardia, dizziness, nasal congestion, miosis .

15. Post-Operatively Observation in ICU Life-long yearly biochemistry tests (Recurrence, Metastasis)

16. Complications Cardiac arrhythmias Dilated cardiomyopathy Heart failure Pulmonary edema Hypertensive encephalopathy Stroke Death