Pheochromocytoma

Pheochromocytoma

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  PHEOCHROMOCYTOMA (PCC) COMPILED BY – MrAshish H. Roy (Nursing Tutor)
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  DEFINITION  Pheochromocytoma isa 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.
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  INCIDENCE  It isnot 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.
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  ETIOLOGICAL CAUSES/ RISKFACTORS  Idiopathic (unknown)  Heredity  Thyroid carcinoma  Parathyroid hyperplasia or tumour  Hemangiomas of the retina, cerebellum, brain stem, spinal cord  Pancreatic cyst  Renal cysts, adenomas  Carcinoma
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  Pathophysiology
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  CLINICAL FEATURES/ SIGNS& SYMPTOMS  5 ‘H’. Hypertension, Headache, Hyperhydrosis, 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
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  Diagnostic Assessment  Historytaking 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 Blood 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 Magnetic Resonance Imaging (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
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  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
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  Surgical Management  Adrenalectomy-unilateral or bilateral.  Laparoscopic removal of the tumor  Laparotomy Selective resection of the tumors, sparing the adrenal cortex
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  NURSING DIAGNOSIS  Painrelated 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.
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  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
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  Post Operative Care 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.
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  Complications  Hypertensive retinopathy Hypertensive nephropathy  Myocarditis ( inflammatory cardiomyopathy)  Increased platelet aggregation  CHF,MI, Arrhythmias, Shock  Renal failure  Aortic aneurism  Fluid and electrolyte imbalance
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  FAQ WHAT ARE CHROMAFFINCELLS ??? 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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  THANKYOU FOR YOURACTIVE LISTENING AND ATTENTION.. IF ANY QUERY REGARDING THE TOPIC KINDLY ASK…. The End.