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Pheochromocytoma Presented by Susie Clabots, Linnea Cooper

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Pheochromocytoma Presented by Susie Clabots, Linnea Cooper, and Stephen Coots Case study, #3 Some people experience paroxysmal, or sudden, periodic attacks of HTN ... – PowerPoint PPT presentation

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Title: 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/

4
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

5
  • 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

7
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/

8
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
10
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)

11
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

16
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

17
So you have an Adrenal Mass?
18
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)

19
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

20
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)

21
Attacks due to.
  • SNS stimulation
  • Accompanied by anxiety.
  • Provoked by some medications antihypertensives,
    opiods, contrast dye, tricyclic antidepressants
  • Lasts minutes to hours

22
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.

23
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?

24
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.

25
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).

26
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)

27
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)

28
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)

29
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

30
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)

31
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

32
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

33
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.

34
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

35
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

36
1
(Onusko 2003)
37
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?

38
Case study 5.
  • How did he handle surgery?
  • Family here?
  • What drugs did you give him?
  • For BP
  • Potential interactions of anesthesia and drugs

39
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)

40
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

41
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

42
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

43
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

44
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

45
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.
    Retrieved April 26, 2008 from http//www.nlm.nih.g
    ov/medlineplus/endocrinediseases.html.
  • Banakik, J. Copstead, L. (2005).
    Pathophysiology, 3rd Edition. St. Louis,
    Missouri Elsevier, Inc.
  •  Ackley, B. Ladwig, G. (2006). Nursing
    Diagnosis Handbook A Guide to Planning Care, 7th
    Edition. St. Louis, Missouri Mosby, Inc.
  •  Graber OBrien, P., Ruff Dirksen, S., Lewis, S.
    L., McLean Heirkemper, M., Bucher, L. (2007).
    Medical-Surgical Nursing Assessment and
    Management of Clinical Problems, 7th Edition. St.
    Louis, Missouri Mosby Elsevier, Inc.
  • Onusko, Edward. (2003). Diagnosing Secondary
    Hypertension. American Family Physician.
    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
    challenges in diagnosis and nursing care. Nursing
    Clinics of North American. 42(1), 101-11.
  • Daub, K. F. (2002, March) Pheochromocytoma, up
    close and personal. Nursing. 32(3), 32hn1-32hn4.
    Retrieved April 27th, 2008 from Proquest Nursing
    and Allied Health Source.
  • Gavaghan, M. (1997, June) Surgical treatment of
    pheochromocytomas. AORN Journal, 65(6) 1039-62.
    Retrieved April 27th, 2008 from GaleHealth
    Reference Center Academic.
  • Jones, SG (1982, February) Adrenal patients.
    Kid-glove care in pheochromocytomapart 2. RN
    45(2), 66-8. Retrieved April 27th, 2008 from
    CINAHL with Full Text
  • Mastropietro, C (1985, February) Anesthesia and
    pheochromocytoma. AANA Journal, 53(1), 60-9.
    Retrieved April 27th, 2008 from CINAHL with Full
    Text.
  • Shen, W.T., Lee, J., Kebebew, E., Clark, O.H.,
    Quan-Yang, D (2006, August) Selective Use of
    Steroid Replacement After Adrenalectomy Lessons
    from 331 Consecutive Cases. Archives of Surgery.
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48
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)
    Pheochromocytoma Treatment. Retrieved April 27th,
    2008 from National Cancer Institute. Web site
    http//www.cancer.gov/cancertopics/pdq/treatment/p
    heochromocytoma/HealthProfessional/page1
  • The Endocrine System How it Functions. Retrieved
    from http//www.youtube.com/watch?vrS7SM4vzs18
    on April 27, 2008
  •  Pecak, Karel. (2008). Management of
    Pheochromocytoma. Retrieved April 26, 2008 from
    http//www.endotext.org/adrenal/adrenal34/ch01s07.
    html
  • Mayo clinic staff. Pheochromocytoma. April 3,
    2007. As retrieved from www.mayoclinic.com on
    April 26, 2008.
  • eMedicine. (2007). Adrenal Insufficiency and
    Adrenal Crisis. Retrieved on April 27, 2008 from
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