Pheochromocytoma

Compiled By: Mr. Ashish Roy

1. PHEOCHROMOCYTOMA (PCC) COMPILED BY – Mr Ashish H . Roy (Nursing Tutor)

2. DEFINITION Pheochromocytoma is a rare condition characterized by a tumor arising from the chromaffin cells of the adrenal medulla that produces excessive catecholamine (epinephrine & nor- epinephrine ) OR A chromaffin cells of the adrenal medulla causing increased secretion of epinephrine or non- epinephrine resulting in hypertension. It is catechdamine secreting neoplasm.

3. INCIDENCE It is not common . It can occur at any age, peak between 40 & 50 years . It can occur at any group age but is most common between the age of 30 and 60. Most pheochromocytoma tumors are benign, 10% are malignant with metastasis . Around 25% of patients have an inherited condition.

4. ETIOLOGICAL CAUSES/ RISK FACTORS Idiopathic (unknown) Heredity Thyroid carcinoma Parathyroid hyperplasia or tumour Hemangiomas of the retina, cerebellum, brain stem, spinal cord Pancreatic cyst Renal cysts, adenomas Carcinoma

5. P athophysiology

6. CLINICAL FEATURES/ SIGNS & SYMPTOMS   5 ‘H’. Hypertension, Headache, H yperhydrosis , Hyper metabolism, Hyperglycemia. Orthostatic hypotension Tachycardia Chest pain ECG changes Heat intolerance Sweating Fever Weight loss Nausea, vomiting Increased blood glucose level Vertigo , diarrhea, polyuria seizures Fatigue Abdominal pain constipation

7. Diagnostic Assessment History taking and physical assessment:- Pheochromocytoma is suspected when the patient shows the signs characterized by 5 ‘ H’.Hypertension , headache, hyperhydrosis , hypermetabolism , hyperglycemia . 24hr urine test Increased B lood Plasma catecholamines ( normal epinephrine- 100pg/ml. nor epinephrine- 100-550pg/ml) Urinary catecholamine's metabolites increased VMA ( vanillylmandelic Acid), normal : 10-250mg/24hr . Glucagons stimulation test CT scan and M agnetic R esonance I maging (MRI) of the adrenal glands or of the entire abdomen are done to identify tumor. Clonidine suppression test is used to distinguish essential hypertension from pheochromocytoma. Radionuclide studies localize the tumor

8. Medical Management Alpha- adrenergic blockers e.g.- phenoxybezamine (10mg-20mg every 6-8hrs) or Prazosin (2-5 mg twice a day) Calcium channel blockers e.g.- nifedepine Beta blockers e.g.- Propanolol Saline & blood products if needed Alpha- Methyl- P- tyrosine if surgery is contra indicated Chemotherapy e.g.- 5 Fluorouracil

9. Surgical Management Adrenalectomy - unilateral or bilateral. Laparoscopic removal of the tumor Laparotomy Selective resection of the tumors, sparing the adrenal cortex

10. NURSING DIAGNOSIS Pain related tumor AEB verbalization Ineffective breathing pattern Related dyspnea/ tachypnea AEB respiratory rate. Activity intolerance Related dyspnea AEB inability to meet ADL. Fluid volume deficit Related diarrhea/ polyuria AEB skin turgor. Ineffective therapeutic regimen Related lack of knowledge of long term management aEB questions about disease. Anxiety related to symptoms from increase osteocholamines headache, palpitation, sweating, nervousness. Risk For ineffective tissue perfusion related to hypotension during postoperative period.

11. Pre- operative Management Check vital signs Assess the level of anxiety Provide supportive care Prepare the patient for tests and surgery Cardiac monitoring Limit activities like bending & lifting Maintain nutritional status with a high protein calorie diet with adequate vitamins & minerals. Administer IV fluids, blood transfusion if needed. Hormonal therapy as prescribed Correct fluid & electrolyte imbalance. Nurse should discuss the patient activites that promote relaxation and stress relaxation techniques Nurse will educate the patient about regarding life style changes that help to decrease number of triggers

12. Post Operative C are Monitoring to detect complications of surgery and Adrenal crisis Blood pressure alteration Blood glucose alteration Fluid & electrolyte imbalance plan the activity & period of rest Assess effects of posture on blood pressure Provide measures to minimize effects of postural hypotension( Orthostatic hypotension - also called postural hypotension - is a form of low blood pressure that happens when you stand up from sitting or lying down. ) Assist the patient during ambulation while BP remains stable Provide measures to decrease the risk of infection in the immuno suppressed patients.( strict surgical asepsis, coughing & deep breathing exercises, avoiding contact with persons with URI) Administer IV fluids as prescribed Monitor serum electrolytes daily, blood glucose levels 4hrly, weight daily, hpurly intake & output. Provide medications as prescribed by the surgeon.

13. Complications Hypertensive retinopathy Hypertensive nephropathy Myocarditis ( inflammatory cardiomyopathy) Increased platelet aggregation CHF,MI , Arrhythmias, Shock Renal failure Aortic aneurism Fluid and electrolyte imbalance

14. FAQ WHAT ARE CHROMAFFIN CELLS ??? Chromaffin cells, also pheochromocytes , are neuroendocrine cells found mostly in the medulla of the adrenal glands in mammals. These cells serve a variety of functions such as serving as a response to stress, monitoring carbon dioxide and oxygen concentrations in the body, maintenance of respiration and the regulation of blood pressure. They are in close proximity to pre-synaptic sympathetic ganglia of the sympathetic nervous system, with which they communicate, and structurally they are similar to post-synaptic sympathetic neurons. In order to activate chromaffin cells, the splanchnic nerve of the sympathetic nervous system releases acetylcholine, which then binds to nicotinic acetylcholine receptors on the adrenal medulla. This causes the release of catecholamines . The chromaffin cells release catecholamines : ~80% of adrenaline and ~20% of noradrenaline into systemic circulation for systemic effects on multiple organs, and can also send paracrine signals. Hence they are called neuroendocrine cells.

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