Davao Doctors College, Inc.

College of Allied Health Sciences

Nursing Program

Concept Mapping
Pheochromocytoma
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College of Allied Health Sciences
Nursing Program
Davao Doctors College, Inc.
College of Allied Health Sciences
Nursing Program

Narrative

Pheochromocytoma, a neuroendocrine tumor, originates from chromaffin cells

that produce vital hormones and are situated in the adrenal glands located atop the
kidneys in the upper abdomen. Approximately 80-85% of these tumors develop
within the adrenal medulla, while 15-20% arise outside this region, termed
extra-adrenal pheochromocytomas or paragangliomas. The majority of
pheochromocytomas are non-cancerous, with only around 10% exhibiting metastatic
tendencies (Grossman, 2024).

People who have certain rare genetic conditions have a higher risk of
pheochromocytomas. Inherited syndromes and genetic changes significantly
contribute to the development of pheochromocytomas and paragangliomas,
accounting for up to 40% of cases. These tumors may arise from certain gene
mutations even without known hereditary syndromes. Specifically, conditions like
Multiple endocrine neoplasia type 2 (MEN2) lead to pheochromocytomas in about
half of affected individuals, while Von Hippel-Lindau syndrome (VHL) results in
tumors in 10% to 20% of cases, often affecting both adrenal glands. Additionally,
Neurofibromatosis type 1 (NF1) is linked to these tumors in approximately 1% to
13% of affected individuals. Mutations in the Succinate dehydrogenase (SDH) gene
family, known as SDHx syndromes, also elevate the risk by up to 45%, depending on
the specific mutation (Mayo Clinic, 2024). Pheochromocytomas may manifest at any
age, with a peak incidence occurring between 40 and 50 years, affecting both
genders equally. Approximately 10% of these tumors are bilateral, and 10% are
malignant. Given the familial clustering of pheochromocytomas, family members of
affected individuals should be informed and screened for this tumor. Notably,
pheochromocytoma may occur as part of familial multiple endocrine neoplasia type
2, warranting consideration in patients with medullary thyroid carcinoma or
parathyroid hyperplasia or tumors (Hinkle & Cheever, 2022).

Pheochromocytoma happens when there is a tumor arising from chromaffin

cells, typically found in the adrenal medulla but can also occur in extra-adrenal
locations, such as sympathetic ganglia (termed paragangliomas) (Grossman, A.,
2024). Chromaffin cells are specialized neuroendocrine cells responsible for
synthesizing and releasing catecholamines. Pheochromocytomas increase the
activity in adrenal medulla that produce and release excessive amounts of
catecholamines, particularly epinephrine and norepinephrine (Gupta PK, & Marwaha
B., 2023). These catecholamines are normally released in response to stress and
play a crucial role in the body's fight-or-flight response. However, in individuals with
pheochromocytoma, the tumor cells continuously secrete catecholamines
independent of external stimuli. Epinephrine and norepinephrine bind to α-adrenergic
receptors in blood vessels, causing vasoconstriction and increasing peripheral
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vascular resistance (Cleveland Clinic, 2022) . This results in hypertension, which is a

hallmark feature of pheochromocytoma. The hypertension associated with
pheochromocytoma can be paroxysmal, characterized by sudden and severe spikes
in blood pressure. These episodes can be triggered by physical activity, emotional
stress, or other factors that stimulate catecholamine release. In addition to
hypertension, excessive catecholamines can lead to other cardiovascular
manifestations such as palpitations, tachycardia, and arrhythmias. These effects
result from the stimulation of β-adrenergic receptors in the heart, leading to
increased cardiac output and contractility (Orr, et. al., 2019). Catecholamines also
have metabolic effects, including increased glycogenolysis and lipolysis, leading to
elevated blood glucose levels and free fatty acids. Patients with pheochromocytoma
may experience symptoms such as diaphoresis (excessive sweating), tremors, and
weight loss due to these metabolic changes. Excessive catecholamines can manifest
as neurological symptoms such as headache, dizziness, and anxiety. Patients may
also experience panic attacks, nervousness, and a sense of impending doom due to
sympathetic nervous system activation.

According to Zerwekh (2023), increase in urinary excretion of total free

catecholamine, vanillylmandelic acid, and metanephrine are seen in urine tests in
patients with pheochromocytoma. Due to an increase in catecholamine, patients will
also develop paroxysmal hypertension that will lead to palpitations, diaphoresis,
nervousness, and headache. According to Grossman (2024), paroxysmal
hypertension is prevalent among most individuals with pheochromocytoma. Ronen
(2019) also stated that when there is an excess of catecholamines, it can lead to a
decrease in insulin release, which in turn can result in an increase in blood sugar
levels. Zerwekh (2023) stated that patients should avoid foods such as coffee, tea,
chocolate, vanilla and aspirin for 2-3 days before the test and during testing of VMA.
They will also have a 24-hour urine collection to test the level of catecholamine. MRI
and CT scan are the imaging tools for determining the location of the tumor
(Grossman, 2024).

According to Patel, D. (2020), for patients with pheochromocytomas,

minimally invasive (laparoscopic or robotic) adrenalectomy is the recommended
course of action. Transabdominal (TA) or posterior retroperitoneoscopic (PRA)
approaches can be used to perform this surgical procedure.

Prior to an operation or as a pharmacologic management, drugs like beta-,

alpha-, and calcium channel blockers maintain the small veins and arteries open and
relaxed. As a result, blood pressure drops and blood flow improves. Additionally,
some of these medications may make the heart beat less forcefully and more slowly.
This can further reduce blood pressure. The first line of treatment for
pheochromocytoma involves α-blockers. Once sufficient α-blockade has been
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achieved, the patient can receive β-blockers to control their heart rate. Although
prazosin, terazosin, and doxazosin can also be used, phenoxybenzamine is the
preferred α-blocker. Calcium channel blockers (CCBs) alone can be used as a
stand-in for α- and β-blockers in low-risk patients. Plus, CBs do not interfere with
assays measuring plasma normetanephrine. In patients with only intermittent
hypertension, CCBs are primarily used to prevent α-blocker-induced sustained
hypotension, to supplement α-blockers in patients with inadequate blood pressure
control, to replace α-blockers in patients with severe side effects, and to avoid having
to increase α-blocker dosages (Garg et al., 2011).

It is critical that a nurse caring for a patient with pheochromocytoma

understands the common symptoms and indicators associated with this condition,
how to diagnose it, how to administer nursing interventions, and how to educate the
patient. Vital organs may sustain damage if blood pressure is excessively elevated
for an extended length of time. Headache, altered vision, altered neurology,
convulsions, and dyspnea are some possible warning signs and symptoms of this.
Alpha-adrenergic blockers are drugs that lower catecholamines by preventing
noradrenaline from being released. These drugs assist in lowering blood pressure
and averting a hypertensive emergency while undergoing surgery. Additionally,
patients are advised against using stimulants because they may cause
vasoconstriction (National Cancer Institute, 2016).

Pheochromocytomas may cause irreversible hypertension, heart failure,

arrhythmias, hypertensive cardiomyopathy, and organ damage if left untreated. They
can also cause life-threatening hypertension and other cardiovascular problems.

After successful removal of a pheochromocytoma, significant improvements

occur in patients' health. The excessive production of catecholamines usually results
in an elevation of blood pressure, which then returns to normal or nearly normal
levels. This lowers the chance of long-term cardiovascular problems like heart
attacks and strokes in addition to lowering the immediate risks. Furthermore, there is
significant relief when the pheochromocytoma-related symptoms disappear.
Treatment reduces or eliminates symptoms such as headaches, sweating,
palpitations, anxiety, and tremors. The prognosis improves with treatment. People
with the condition can often be cured by having the tumor surgically removed.
However, some patients may experience high blood pressure even after undergoing
surgery. Medication is an option for treating this. Following surgery, there is a chance
that the tumor may recur. Years following the initial course of treatment, this can
occur. Owing to this risk, individuals who have received treatment for
pheochromocytoma ought to undergo routine examinations to track any potential
recurrence of the illness.
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In summary, Pheochromocytoma involves genetic predisposition,

dysregulated catecholamine production, and widespread activation of the
sympathetic nervous system. This leads to a broad spectrum of clinical
manifestations, including hypertension, cardiovascular symptoms, and metabolic
effects. Prompt diagnosis and management are crucial to prevent potentially
life-threatening complications associated with this condition.
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References

Cleveland Clinic. (2022). Pheochromocytoma: Causes, Symptoms & Treatment.

https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

Garg, M., Kharb, S., Brar, K. S., Gundgurthi, A., & Mittal, R. (2011). Medical
management of pheochromocytoma: Role of the endocrinologist. Indian
Journal of Endocrinology and Metabolism, 15(8), 329.
https://doi.org/10.4103/2230-8210.86976

Grossman, A. (2024). Pheochromocytoma - Endocrine and Metabolic Disorders.

https://www.msdmanuals.com/professional/endocrine-and-metabolic-disorder
s/adrenal-disorders/pheochromocytoma

Grossman, A. B. (2024, March 28). Pheochromocytoma. MSD Manual Professional

Edition.
https://www.msdmanuals.com/professional/endocrine-and-metabolic-disorder
s/adrenal-disorders

Gupta PK, & Marwaha B. (2023). Pheochromocytoma. In: StatPearls [Internet].

Treasure Island (FL): StatPearls Publishing; 2024 Jan-.
https://www.ncbi.nlm.nih.gov/books/NBK589700/

Hinkle, J.L. & Cheever, K.H., Overbaugh, K.J. (2022). Brunner & Suddarth's
Textbook of Medical-Surgical Nursing (15th ed.). Philadelphia: Wolters Kluwer.

Mayo Clinic. (2024). Pheochromocytoma - Symptoms and causes.

https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/symptom
s-causes/syc-20355367

Msn, J. Z. (2015). Illustrated Study Guide for the NCLEX-RN® Exam. Elsevier Health
Sciences.
http://books.google.ie/books?id=hxzmCgAAQBAJ&printsec=frontcover&dq=Z
erwekh,+J.+(2023).+Study+Guide+for+the+NCLEX-RN+Exam+(11th+ed.).+El
sevier.&hl=&cd=1&source=gbs_api

National Cancer Institute. (2016). Pheochromocytoma and Paraganglioma

Treatment.
https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma
-treatment-pdqdoi.org
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Orr, WB., Chokshi, R., Groh, G., Silva, JNA., Van Hare, GF., & Dalal, AS. (2019). A
unique cardiovascular presentation of pheochromocytoma. SAGE Open
Medical Case Reports. 2021;9. doi:10.1177/2050313X21994037

Patel, D. (2020). Surgical approach to patients with pheochromocytoma. Gland

Surgery, 9(1), 32–42. https://doi.org/10.21037/gs.2019.10.20

Zerwekh, J. (2023). Illustrated Study Guide for the NCLEX-RN® Exam (11th ed).
Elsevier Health Sciences.

GROUP MEMBERS & ASSIGNED TASK

Jamero, Heraleen - Disease Process, Narrative, and Compile

Jover, Eula Marie - Management and Narrative

Kobayashi, Princess - Disease Process, Narrative and Edit

Ladao, Sherry Lois - Etiology, Prognosis, and Narrative

Laput, Daniela John Mary - Signs and Symptoms, Diagnostic tools, and Narrative