Pheochromocytoma Presented by Susie Clabots, Linnea Cooper

1
PheochromocytomaPresented by Susie Clabots,
Linnea Cooper, and Stephen Coots
2
Pheochromoctoma What is it?

• Tumor in the medulla of the adrenal gland.
• Mostly benign
• Often produces large amounts of catecholamines.
• Reported number of tumors vary widely. Occurs in
  approximately 1 in 1000 people.
• Estimated 800 new cases annually

(Mastropietro 1985)
3
Endocrine Glands and Hormones

• Posterior pituitary
• Oxytocin- milk let down, contractions during
  childbirth
• ADH- water balance
• Pituitary- MSH
• Thyroid
• T3, T4, Calcitonin
• Parathyroid
• PTH
• Hypothalamus controls hormone production in
  pituitary
• http//www.hormone.org/Endo101/

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Adrenal glands

• Outer- cortex
• Glucocorticoids
• Control blood sugar, burning of protein and fat,
  response to stress like illness or injury
• Mineralcorticoids
• Aldosterone control blood volume, regulate blood
  pressure by working on kidney
• Activation of R-A-A, as a result of dec blood
  flow to kidney, releases angiotension II
• Elevated potassium/low sodium leads to
  aldosterone secretion
• Androgens
• Growth and development in both sexes
• Stimulate pubic and axillary hair growth, sex
  drive in females

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• Inner- medulla
• Produces epinephrine, norepinephrine, dopamine
• Catecholamines are hormones when secreted by
  medulla but neurotransmitters when secreted by
  nerve cell.
• www.mayoclinic.com

6
Cortisol

• Increases blood glucose
• stimulation of hepatic gluconeogenesis
• inhibiting protein synthesis
• stimulate lipolysis in adipose tissue
• Decrease peripheral glucose in fasting state.
• Anti-inflammatory action
• Increase cortisol during times of stress to more
  effectively deal with it.
• Alter cell-mediated response

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Negative feedback

• Endocrine system is regulated primarily by
  negative feedback.
• Glands respond by increasing or decreasing the
  amount of hormone produced based on the feedback
  it gets from body signals.
• When the levels of these hormones get to a
  certain level, the release of the hormones is
  shut off.
• http//www.hormone.org/Endo101/

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Changes in the elderly

• Inc secretion of norepinephrine, no change in
  epinepherine.
• Decreased B-adrenergic receptor response to
  norepi.
• Dec responsiveness to medications that work on
  b-receptors
• May account for increased
• prevalence of hypertension
• with aging
• (Graber-Obrien 2007)

9
Chromaffin Cell tumor

• Chromaffin Cells are found in the adrenal medulla
  and the ganglia of the sympathetic nervous
  system.
• Cat release not initiated by neural impulses
• They secrete norepinephrine, epinephrine,
  dopamine.

A Primer on Chromaffin Cells http//webpages.ull.e
s/users/isccb12/ChromaffinCell/Primer.html
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Who gets it?

• Less often diagnosed in African American
• Equal occurrence in females and males
• Can occur at any age. Most common in people
  between 30 and 60
• Usually on Right side
• Incidence decreases with age(Hammer 2006)

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Associated with..

• Multiple endocrine neoplasia, type II (MEN-II)
• Thyroid cancer (medullary)
• Von Hippel-Lindau disease
• Rare multi-system disorder at higher risk for
  developing
• Neurofibromatosis
• Includes multiple tumors in the
  skin(neurofibromas), pigmented skin spots, tumors
  of optic nerve, and bone lesions. Higher risk of
  having pheo.
• www.mayoclinic.com

12
Von Hippel Lindau Syndrome (VHL)

• VHL tumor suppressor protein helps protect the
  body from tumors. When this protein is rendered
  inactive by a germline mutation malignant and
  benign tumors are more likely to occur, including
  pheo.

www.emedicine.com
13
Neurofibromatosis

• Autosomal dominant disorder
• Comes in many different forms (eight or more
  phenotypes and at least two different genetic
  disorders)
• Can effect the skin, bones, nervous system, and
  endocrine system.
• Most commonly effects the skin.
• Increased chance of pheo

www.emedicine.com
14
Multiple Endocrine Neoplasia (MEN)

• Definition Two endocrine neoplasia tumor types
  occur in the body with evidence of either a
  causative mutation or hereditary transmission.
• Occurs by a mutation that renders MENIN, a tumor
  suppressor protein, inactive or by mutations to
  the RET gene
• Occurs twice as often in men as women.
• Genetic disorders account for about 10 of pheos

www.emedicine.com
15
Malignant Pheochromocytoma

• 3-36 of pheochromocytomas
• Location of metastasis predicts survival
• Lung and liver worst, bone best
• 5 year survival 34-60
• Less than 40 respond to therapy, reduction in
  size lessens symptoms only
• Chemotherapy should be aware of potentially
  fatal complications arising from excessive
  catecholamine release as tumor cells are
  destroyed (usually within the first 24 hr).
• http//www.endotext.org/adrenal/adrenal34/ch01s07
  .html

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Pheo Outside the Adrenal Glands

• About 10 of pheochromocytomas occur outside the
  adrenal glands. They are referred to as
  Paragangliomas (also extra-adrenal
  pheochromocytomas).
• Very rarely malignant.
• Similar symptoms to pheochromocytomas.
• Some extra symptoms depending on the location of
  the tumor.
• About 85 of paragangliomas occur in the abdomen

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So you have an Adrenal Mass?
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Clinical manifestation

• Severe episodic hypertension
• Accompanied by severe, pounding HA of sudden
  onset, tachycardia/arrythmias, profuse sweating,
  anxiety, palpitations, unexplained abd or chest
  pain, pallor, weight loss, fever, hypertensive
  retinopathy, nausea, weakness, flushing and
  constipation.
• The syndrome can vary depending on the types of
  catecholamines being produced, the amount and
  frequency of their release into the circulation,
  and other factors (Onusko 2003).
• Massive catecholamine release can be induced by
  positional change
• Average dec 14mmHg in pt opposed to 4mmHg in pt
  with essential HTN (Hammer 2006)

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Hypertensive Crisis

• Life-threatening, compromise vital organs
• Results from increase in catecholamines from the
  tumor.
• BP gt180/110
• Blood vessels become inflamed and leak fluid
• Heart not able to keep up with demand
• Leads to coronary artery disease, left
  ventricular hypertrophy, heart failure
• www.mayoclinic.com

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Differential Dx

• Pt with pheo have mildly elevated baseline BP
• present with HTN crisis with BPs 200/110 followed
  by severe exhaustion
• HTN not labile
• unable to equate it with stress or emotional
  distress
• Panic Attacks
• Mildly elevated BP
• Feeling of doom is primary sign
• (Hammer 2003)

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Attacks due to.

• SNS stimulation
• Accompanied by anxiety.
• Provoked by some medications antihypertensives,
  opiods, contrast dye, tricyclic antidepressants
• Lasts minutes to hours

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Case study 1

• 1. The Physician informs T.L. that an adrenal
  tumor is causing his symptoms. T.L. is obviously
  upset with his diagnosis. He states he doesnt
  understand how a tumor on top of his kidney can
  cause high BP. He asks whether this means he has
  cancer. How would you respond?
• Most pheo are benign and can be removed. Only
  6-10 of pheos are cancerous and metastasize.
  Typically, they are limited to one local area and
  will not spread to other parts of the body.
• Malignant (cancerous) tumors spread to
  neighboring tissue and blood vessels and can
  metastasize to other parts of the body.

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Case study 2

• The physician advises T.L. to undergo an
  adrenalectomy. He is immediately started on
  phenoxybenzamine (Dibenzyline) 10mg PO g12h.
  This medication is titrated up q3d until T.L.s
  supine BP is below 160/90 mmHg and his standing
  BP is above 85/40. Propranolol (Inderal) 20mg PO
  qid is added to control his tachyarrhythmia.
  What is the connection between these two drugs
  and the diagnosis?

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Phenoxybenzamine (Dibenzyline)

• Class long acting alpha adrenergic blocker
• Indications Management of blood pressure
• Action relaxes smooth muscle, causes BP to
  decrease
• Dosage initially 10 mg twice daily. Can be
  increased every other day, usually to 20-40 mg 2
  or 3 times a day.
• Adverse side effects Dizziness, drowsiness,
  fainting, lightheadedness. (Alcohol, hot weather,
  exercise, and fever can increase these effects.).
  Postural hypotension, tahcycardia Also, pt
  should sit up or stand slowly, especially in the
  morning. Sit down at first sign of dizziness or
  weakness.
• Interactions Potentiates effect of medicines
  that stimulate both alpha and beta adrenergic
  receptors (i.e. epinephrine) and so exaggerrated
  hypotensive response and tachycardia can occur.
  Dibenzyline can blocks hyperthermia production by
  levarterenol and blocks hypothermia production by
  reserpine.

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Propanolol (Inderal)

• Class Antiarrhythmics
• Pharmacologic beta blockers
• Indications Management of arrhythmias.
  Management of HTN.
• Manage pheochromocytoma.
• Action Blocks stimulation of beta1 (myocardial)
  and beta2 (pulmonary,
• vasular and uterine) adrenergic receptor sites.
  
• Therapeutic effects Decreases HR and BP,
  suppression of
• arrhythmias, prevention of MI.
• Dosage for adjunct management of
  pheochromocytoma 20mg 2 to 3
• times a day concurrently with alpha-blocking
  therapy.
• Adverse effects fatigue, weakness, dizziness,
  drowsiness, impotence,
• arrythmias, bradycardia, CHF, pulmonary edema,
  bronchospasm
• Interactions General anesthesia, IV phenytoin,
  and verapamil (can cause
• additive myocardial depression). Additive
  hypotensive effects with other antihypertensives,
  acute ingestion of alcohol, or nitrates.
  Concurrent use of amphetamines, cocaine,
  ephedrine, epi, norepi, may result in unopposed
  alpha-adrenergic stimulation (excessive HTN,
  bradycardia).

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Diagnostic Procedures

• Only 0.1 of HTN
• Plasma Metanephrine (High sensitivity, lower
  specificity)
• gt236 ng/L confirms dx
• Measurement of urinary fractioned metanephrines
  (catecholimine metabolites) as well as
  fractionated catecholamines and creatinine
• Done as 24 hr urine
• Sometimes tumor doesnt produce enough
  catecholamines to elicit a reading
• SNS nerve fibers also produce catecholamines
• Vanillylmandelic acid (VMA) 24 hr urine, less
  sensitive
• Plasma catecholamines/stimulation test
• CT/MRI for tumor location
• Clonidine suppression test
• (Hammer 2003)

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Plasma catecholamines collection

• Drugs, diet and stress can all raise levels
• Acetaminophen interferes- stop taking 5 days
  prior.
• Caffeine, nicotine interfere with results
• Emotional stress interferes
• Interfering medications should be stopped 2 weeks
  prior tricyclic antidepressants, antipsychotics,
  levadopa, ethanol, phenoxybenzamine, withdrawing
  from clonidine
• Supine position for draw, after night fast
• (Hammer 2003)

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Labs drawn

• Routine urinalysis,
• Complete blood cell count,
• blood chemistry profile (potassium, sodium,
  creatinine, fasting glucose, fasting lipid
  levels),
• 12-lead electrocardiogram are recommended for
  all patients with hypertension.
• (Onusko 2003)

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Treatment

• Surgical removal of tumor
• Laparoscopic adrenalectomy
• If surgery not option metyrosine used to
  diminish catecholamine production and simplify
  chronic management
• Preoperatively alpha-adrenergic blockers
• Sympathetic blocking agens (minipress) HTN and
  other symptoms
• B-blockers

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Laproscopic Transperitoneal Surgery

• Currently standard of practice
• 2 incision
• Reduced post op pain
• Reduced length of stay
• Earlier return to regular activities
• Decreased postop complications
• 2/61 patients
• Essential preoperative alpha an beta-blockers and
  tight BP control intraoperatively.
• (Jaroszewski 2003)

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Interventions

• Preoperatively calm environment, rest
• Assess observe for classic triad severe
  headache, tachycardia, profuse sweating.
• Orthostatic hypotension- SE of meds and surgery
• Intraoperative
• monitor for hypovolemia and hypotension
• monitor glucose levels
• Postoperatively
• Case manage refer to health care provider

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Nursing Diagnosis

• Anxiety r/t symptoms from increased
  catelcholamines- headache, palpitations,
  sweating, nervousness, N/V, syncope
• Disturbed sleep pattern r/t high levels of
  circulating catelcholamines
• Ineffective Health Maintenance r/t deficient
  knowledge regarding treatment and self-care
• Nausea r/t increased catelcholamines
• Risk for ineffective tissue perfusion
  cardiopulmonary and renal r/t episodes of
  hypertension

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Anxiety r/t symptoms from increased
catelcholamines- headache, palpitations,
sweating, nervousness, N/V, syncope

• Outcome Pt will have vital signs that reflect a
  baseline or decreased sympathetic stimulation.
• Outcome Pt is able to identify and verbalize
  symptoms and possible causes of anxiety
• Outcome Pt is able to verbalize and demonstrate
  techniques that can help control anxiety.
• Interventions Nurse will assess pts level of
  anxiety and physical reactions to anxiety. To get
  a baseline understanding.
• Nurse can educate pt about signs/symptoms of
  excessive sympathetic stimulation. So pt can
  understand and self-monitor for s/sx.
• Nurse will educate pt regarding lifestyle changes
  that can help decreased the number of triggers.
  (more important pre-operatively, but still
  important post-op, esp. if HTN remains).
• Nurse will discuss with pt activities that
  promote relaxation and also stress reduction
  techniques.
• Nurse will intervene when necessary. Anxiety is
  a normal response to actual or perceived danger
  if the threat is removed, the response will stop.
  So if pt gets sudden severe headache,
  palpitations, diaphoresisthe nurse can attend to
  him and reassure him, explain what is happening,
  get him to sit down.

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Case study, 3

• Some people experience paroxysmal, or sudden,
  periodic attacks of HTN that correspond to the
  release of epinephrine and/or norepinephrine.
  Under what circumstances would T.L. most likely
  experience a paroxysmal hypertensive event?
• At work- his position as a supervisor
• Dont know much about him
• Kids, family? Elderly parents? Job stress?
• Being in hospital
• Paying bill
• Exercise
• Caffeine
• B-blockers and anesthesia can increase secretion
  of catecholamines
• Excessive ingestion of tyrosine-containing foods
  (aged cheese, red wine, beer, yogurt)
• If on MAOI

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Case study 4

• What measures to prevent a paroxysmal
  hypertensive event should you teach T.L.?
• HTN resulting from an identifiable, correctable
  cause
• Avoid tyrosine containing foods (if on MAOI)
• Avoid stress, exercise (until after the surgery),
  palpating adrenal glands
• Meds should be evaluated to see if they aggravate
  his condition
• Stop/limit smoking
• Avoid extreme excitement
• Teach stress reduction techniques/limit work/take
  time outs

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1
(Onusko 2003)
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Case study 5

• Following the surgery, T.L. is taken directly to
  the ICU. The anesthesiologist gives the
  admitting nurse the following report surgery
  went well, and T.L. should wake up shortly his
  VS have been running 180/90, 88, 16, 96.1 F hes
  got a left subclavian Swan-Ganz catheter and two
  large-bore peripheral IVs with D5W running at a
  total of 125mL/h urine output during OR was
  200mL. What additional data should the ICU nurse
  elicit from the anesthesiologist?

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Case study 5.

• How did he handle surgery?
• Family here?
• What drugs did you give him?
• For BP
• Potential interactions of anesthesia and drugs

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Case study, 6

• Identify three postoperative issues R/T T.L.s
  care.
• Blood glucose- can lead to DM
• Monitor
• Proper diet
• After tumor removal, immediately start D5W and
  continue for several hours (Pecak 2008)
• Htn can persist (10-30) stress importance of
  follow up care
• Establish pcp, teach importance, teach s/s htn
  crisis
• Orthostatic hypotension
• Give fluids right away (Pecak 2008)
• Teach to get up slow, safety measures
• SE of many meds
• Good nutrition, sleep
• Control stress
• Pain- post-op
• Biochemical testing should be repeated after
  about 14-28 days from surgery in order to check
  for remaining disease
• On long term follow-up about 17 of tumors recur,
  with about 50 of these showing signs of
  malignancy. (Pecak 2008)

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Case study, 7

• For each issue identified in question 6, outline
  two to three interventions/measures.
• Assess the patient for pain
• Administer pain medication as ordered.
• Administer fluids as ordered.
• Tell the patient youd like to be in the room
  when he gets out of bed.
• Monitor blood pressure frequently.
• Assess neurological status
• Instruct the patient to shift positions slowly
  and give himself time between each shift.
• Teach stress reduction techniques

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Case study 8

• During shift assessment (second post operative
  day), the nurse notes that T.L. seems less alert,
  his grip strength is markedly weaker than
  yesterday, and his mucous membranes are dry. The
  previous nurse reported that he had vomited twice
  in the last hour. The cardiac monitor shows
  peaked T waves and a widened QRS complex. VS are
  120/72, 94, 14, 101 F. What conclusions can you
  draw from the foregoing data?
• All these findings suggest adrenal insufficiency

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Adrenal insufficiency

• Result of removal of adrenal gland
• Aldosterone no longer being released
• Normally acts on kidneys, gut, and salivary/sweat
  glands to affect electrolyte balance
• Cortisol
• Responds to stress, immune system, cv function,
  insulin balance
• s/s Weakness (99)
• Pigmentation of skin (98)
• Weight loss (97)
• Abdominal pain (34)
• Salt craving (22)
• Diarrhea (20)
• Constipation (19)
• Syncope (16)
• http//www.emedicine.com/emerg/topic16.htm

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Case study 9

• What treatment measures would you anticipate for
  adrenal insufficiency?
• Steroid replacement therapy (normally should only
  be used in Cushing syndrome or bilateral
  adrenalectomy)
• Fluids
• Electrolyte replacement
• http//archsurg.ama-assn.org/cgi/content/full/141/
  8/771?cknck

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Case study 10

• Outline four measures that are critical during
  this period.
• Ensure IV line patency and insertion site and
  maintain adequate fluid infusion rate to prevent
  further dehydration from vomiting
• Monitor VS q 1 hr
• Administer antipyretics
• Start steroid therapy per physician

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Case study 11

• T.L. is stabilized and is scheduled to be
  discharged home. During discharge teaching, T.L.
  asks whether he will require medication for the
  rest of his life. How should you respond to
  T.L.?
• Life long therapy is generally needed quality of
  life is able to be maintained.

46
Reference

• Hammer, G and Nguyen-Martin, M (2006).
  Pheochromocytoma An Update on Risk Groups,
  Diagnosis, and Management. Hospital Physician.
  Turner White Communications, Inc Wayne, PA. pp
  17-26.
• The Hormone Foundation (2008). ENDO 101 The
  Endocrine System. Retrieved April 26, 2008 from
  http//www.hormone.org/Endo101/
• Medline Plus (2008). Endocrine Diseases.
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  ov/medlineplus/endocrinediseases.html.
• Banakik, J. Copstead, L. (2005).
  Pathophysiology, 3rd Edition. St. Louis,
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•  Ackley, B. Ladwig, G. (2006). Nursing
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•  Graber OBrien, P., Ruff Dirksen, S., Lewis, S.
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• Onusko, Edward. (2003). Diagnosing Secondary
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  67(1)67-74
• Jaroszewski, Dawn, et al. (2003). Laparoscopic
  Adrenalectomy for Pheochromocytoma. Mayo Clin
  Proc 2003781501-1504

47
References

• Daub, K. F. (2007, March) Pheochromocytoma
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• Jones, SG (1982, February) Adrenal patients.
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References.

• Carmichael, S Winkler, H. A Primer on
  Chromaffin Cells. Retried April 27th, 2008, from
  The Chromaffin Cell Web site http//webpages.ull.
  es/users/isccb12/ChromaffinCell/Primer.html
• National Cancer Institute. (2008)
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  2008 from National Cancer Institute. Web site
  http//www.cancer.gov/cancertopics/pdq/treatment/p
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• The Endocrine System How it Functions. Retrieved
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  on April 27, 2008
•  Pecak, Karel. (2008). Management of
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• eMedicine. (2007). Adrenal Insufficiency and
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