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  1. 1. clinical nursing skills (ACADEMIC FORUM) weekly series presentation on: PHEOCHROMOCYTOMA PREPARED BY COMR. NR. ABDULMALIK UMAR DALA 1
  3. 3. INTRODUCTION • Pheochromocytoma derived from four greek words pheo: which means “dark”, chromo: “color”, cyt: “cell” and oma: tumor. • Pheochromocytoma is a neuroendocrine tumor derived from the chromaffin cells of the medullary portion of the adrenal glands, being characterized by the excessive production and release of catecholamines (adrenaline and noradrenaline). • It is a rare tumor but treatable tumor that forms in the middle of the adrenal gland. • In most cases the tumor is benign, but it can be malignant (cancerous). • The tumor is made by certain types of cell called the chromaffin cell, which produce and release hormones that cause the “fight or flight” response. 3
  4. 4. DEFINITION Pheochromocytoma is defined as a tumor found on the adrenal gland (adrenal medulla) of the kidneys that secretes excessive amounts of catecholamines (adrenaline and noradrenaline). 4
  5. 5. CLASSIFICATIONS OF PHEOCHOROMOCYTOMA Most of the pheochromocytomas are benign (not cancerous ). Approximately about 10% to 15% of the pheochromocytomas are malignant ( cancerous). Staging system of the pheochromocytoma if it’s cancerous There is no standard staging system of the cancerous pheochromocytoma but instead it can be described as the following: 1. Localized pheochromocytoma: the tumor is in one or both of the adrenal gland. 2. Regional pheochromocytoma: the cancer has spread to lymph nodes or other neighboring tissues closed to adrenal gland. 5
  6. 6. CONT. 3. Metastatic pheochromocytoma: the cancer has spread to other parts of the body such as liver, lungs, bone or distant lymphnodes. 4. Recurrent pheochromocytoma: the cancer has recurred (come back) after it has been treated. It might come back in the same place or in another part of the body. 6
  7. 7. What are adrenal glands? • Adrenal glands are part of endocrine system and are located on top of each kidneys. Each adrenal gland has two parts. The outer layer part called the adrenal cortex and inner layer part called the adrenal medulla. • The adrenal medulla (which is the site mostly affected) makes hormones called catecholamines that help to regulate the following important bodily functions and aspect: Heart rate Blood sugar (blood glucose) Blood pressure The way our body respond to stress ( “fight or flight” response) 7
  8. 8. CONT. The primary cetocholamines includes: • Dopamine • Ephinephrine ( adrenaline) • Norephinephrine (noradrenaline) 8
  9. 9. CAUSES AND RISK FACTORS • In most cases of pheochromocytoma the exact causes is idiopathic. • Approximately about 25% to 35% of cases seem to run in families. • The tumors are more common in people who have disorders or conditions that are passed down from parents to children, including: a. Multiple endocrine neoplasia, type II b. Von Hippel-Lindau disease c. Neurofibromatosis 1 (NF1) d. Hereditary paraganglioma syndrome NB: It may also be cause by mutations (changes) of one of at least 10 different genes. 9
  10. 10. PATHOPHYSIOLOGY • Pheochromocytoma occur due to excessive release of catecholamine hormones, secreted by the tumor, continuously or in an episodic manner. • These hormones, which include epinephrine, norepinephrine, and dopamine, excessively stimulate alpha- and beta-adrenergic receptors, with all the clinical manifestations in pheochromocytoma being related to this. • The symptomatology in pheochromocytoma is determine by the predominant type of catecholaminergic secretion. 10
  11. 11. CONT. • Epinephrine stimulates beta-1 and beta-2 adrenergic receptors. By stimulating beta-2 adrenergic receptors, epinephrine causes vasodilation in striate muscles. • Also, epinephrine has metabolic effects, stimulating hepatic glycogenolysis and gluconeogenesis, thus leading to hyperglycemia and block the role of insulin. • Norepinephrine stimulates alpha-1, alpha-2, and beta-2 adrenergic receptors, leading to vasoconstriction and increased blood pressure. 11
  12. 12. CONT. • Epinephrine and norepinephrine have positive chronotropic and inotrophic effects through their action on beta-1adrenergic receptors hence increase the heart rate and the force of contraction leading to increase cardiac output and systolic blood pressure. • Dopamine stimulates dopaminergic receptors D1 and D2. In cases of pheochromocytoma, dopamine concentrations are elevated, thus causing vasoconstriction and increased heart rate. 12
  13. 13. CONT. • The hypertensive crisis may occur which is associated with palpitations and diaphoresis, as a result of the massive release of catecholaminergic hormones from the tumor. • Moreover, pheochromocytoma is characterized by the release of other substances, such as neuron-specific enolase, vasoactive intestinal peptide, or adrenocorticotropic hormone (ACTH), which cause various other clinical manifestations. 13
  14. 14. CLINICAL MANIFESTATIONS  Facial flushing (from hypertension).  Fluttering in chest (palpitations).  Increased blood pressure and heart rate.  High glucose level.  Headaches (sudden and severe).  Tremors. 14
  15. 15. CONT. Frequent sweating (from hypertension). Loss of weight. Increase anxiety and fear. Growing tumor can cause back or abdominal pressure or pain. Heat intolerance. Tired and weak (from the constant stimulation). 15
  16. 16. CONT. The Signs and symptoms can happen in episodes or triggered after certain events such as: • Eating foods with Tyramine (plays a role in pressure blood): foods that are aged, pickled, fermented like cheeses, red wine, smoke/dried meat, bananas, chocolate. • Surgery, trauma injury, emotional stress, medications such as Monoamine Oxidase Inhibitors (MAOIs). 16
  17. 17. DIAGNOSIS Blood or urine tests: to look for high levels of hormones such as catecholamines and metanephrines (are metabolites formed when the body breaks down catecholamines). MRI (magnetic resonance imaging) scan: which uses powerful magnets and radio waves to make images of organs and tissues to check for a tumor CT scan: which puts together several X-rays taken from different angles to spot a tumor. 17
  18. 18. CONT. M-iodobenzylguanidine (MIBG) imaging: a scanning technology that can detect tiny amounts of an injected radioactive compound taken up by pheochromocytomas or paragangliomas. Positron emission tomography (PET): a scanning technology that also can detect radioactive compounds taken up by a tumor. 18
  19. 19. TREATMENTS  Surgery  Radiation therapy.  Chemotherapy.  Ablation therapy.  Embolization therapy.  Targeted therapy. 19
  20. 20. Surgery • The best treatment option of pheochromocytoma is surgery called adrenalectomy. • Adrenalectomy: involves the remove of the adrenal gland with the tumor (may remove one or both depending on where tumor is located). 20
  21. 21. Cont. Preoperative prescription (that is prior to the surgery) • Alpha-adrenergic blockers: such as phenoxybenzamine, doxazosin (Cardura) and prazosin (Minipress) work by blocking noradrenaline, reduces catecholamines. These medications help decrease blood pressure and prevent a hypertensive crisis during surgery. • Beta-adrenergic blockers: like atenolol (Tenormin), metoprolol (Lopressor, Toprol-XL) and propranolol (Inderal, Innopran XL) to help with hypertension and tachycardia. 21
  22. 22. Radiation therapy Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. There are two types of radiation therapy: External radiation therapy: This therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy: This therapy uses a radioactive substance sealed in needles, seeds, wires or catheters that a healthcare provider places directly into or near the cancer. 22
  23. 23. Chemotherapy • Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by killing the cells or by preventing them from dividing and multiplying. • Chemotherapy is usually given intravenously through a vein (intravenously). It’s usually an effective treatment, but it can cause side effects. 23
  24. 24. Ablation therapy • Ablation therapy is a minimally invasive treatment option that uses very high or very low temperatures to destroy tumors. • Ablation therapies that can help kill cancer cells and abnormal cells include: Radiofrequency ablation: This therapy uses radio waves to heat and destroy cancer cells and abnormal cells. The radio waves travel through electrodes (small devices that carry electricity). Cryoablation: This therapy uses liquid nitrogen or liquid carbon dioxide to freeze and destroy cancer cells and abnormal cells. 24
  25. 25. Embolization therapy • Embolization therapy is a pheochromocytoma treatment that blocks the artery leading to your adrenal gland. • Blocking the blood flow to your adrenal glands helps kill the cancer cells that are growing there. 25
  26. 26. Targeted therapy • Targeted therapy is a treatment option that uses medications or other substances to attack specific cancer cells without harming healthy cells. • Healthcare providers use targeted therapies to treat metastatic and recurrent pheochromocytoma. NB: Researchers are currently studying sunitinib, a type of tyrosine kinase inhibitor, for the treatment of metastatic pheochromocytoma. Tyrosine kinase inhibitor therapy is a type of targeted therapy that prevents tumors from growing. 26
  27. 27. NURSING MANAGEMENT Monitor vital signs most especially the blood pressure and heart rate Monitor for hypertensive crisis: >180 systolic or >120 diastolic, if blood pressure is too high for a long period of time this can cause damage to vital organs like kidneys, eyes, brain and heart. Signs and symptoms of this includes: headache, vision changes, neuro changes, seizures, and shortness of breath. Monitor for chest pain (risk for MI), neuro status (stroke), EKG changes, hyperglycemia Provide a calm and cool environment Administer prescribed drugs 27
  28. 28. Cont. Health education for patients with pheochromocytoma includes the following: • Eat high calorie diet: burning fats at a rapid rate. • Avoid stimulant substances: energy drinks, caffeine products, or smoking (due to vasoconstriction). • For patient going for an adrenalectomy: educate about having to take hormone replacement medications after surgery and taking alpha-adrenergic prior to surgery (usually 2 weeks before surgery). • If patient is having a bilateral adrenalectomy (both glands removed): will have to take glucocorticoids and mineralocorticoid for life. • If patient is having a unilateral adrenalectomy (only one gland removed): will have to take glucocorticoids for approximately 2 years. 28
  29. 29. NURSING DIAGNOSIS • Risk of altered systemic tissue perfusion related to fluctuation in cardiovascular system status. • Anxiety related to increase in circulating of catecholamine. • Altered nutrition related to increase metabolic rate. • Risk of altered health maintenance related to insufficient knowledge of follow up care. 29
  30. 30. COMPLICATIONS • Heart muscle disease (cardiomyopathy) • Heart muscle inflammation (myocarditis) • Brain bleeding. • pulmonary edema • Stroke. • Heart attack. • Kidney failure. • Nerve damage most especially eyes. 30
  31. 31. REFERENCES • Dahia PL. Pheochromocytoma and paraganglioma pathogenesis: Learning from genetic heterogeneity. Nat Rev Cancer. 2014; 14(2): 108–19. • Bravo E, Tagle R. Phaeochromocytoma: state-of-the- art and future prospects. Endocrine Reviews. 2003; 24(4): 593-53. • Galetta F, Franzoni F, Bernini G, et al. Cardiovascular complications in patients with pheochromocytoma: a mini-review. Biomed Pharmacotherapy. 2010; 64(7): 505-9. • Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014; 99(6): 1915–42 31
  32. 32. Clinical nursing skills (ACADEMIC FORUM) THANK YOU FOR READING Prepared by comr. Nr. Abdulmalik umar dala FOR MORE INFOR CONTACT THE PRESENTER AT: PHONE NUMBER: 08165385343 EMAIL: nursinghealthconsult@gmail.com