Pheochromocytoma is a rare neuroendocrine tumor that originates from chromaffin cells of the adrenal medulla. It secretes high amounts of catecholamines, mostly epinephrine and norepinephrine. Symptoms include hypertension, headache, sweating, anxiety and weight loss. Diagnosis involves measuring catecholamines in blood and urine samples. Imaging tests like CT, MRI, MIBG scan and PET are also used. Preoperative treatment includes alpha-blockers to lower blood pressure. Surgical removal of the tumor is the primary treatment. Postoperative care focuses on blood pressure monitoring and managing stress.

1. PHEOCHROMOCYTOMA
Prepared by: Roxanne Mae E. Birador S.N.

2. PHEOCHROMOCYTOMA
A neuroendocrine tumor of
the medulla of the adrenal
glands (originating in the chromaffin
cells) and secretes high amounts
of catecholamines, mostly epinephrine,
plus norepinephrine to a lesser extent.
phaios "dark“
chroma "color“
kyto s "cell“
-oma "tumor“

3. Worldwide, the prevalence of
this disease is 2 to 8 per million
cases, but may occur frequently in
certain populations.
In the Philippines, a hospital
census done during the 2009,
showed that only four of 101 cases
of secondary hypertension were
found to have pheochromocytoma.

4. In the Philippine General Hospital, only four
patients have biopsy proven
Pheochromocytoma during the past five years.

5. Most people with a
pheochromocytoma are between the ages of 40 and
50, but the tumor can develop at any age.

6. No known environmental, dietary, or lifestyle risk factors have been
linked to the development of pheochromocytoma.
•Multiple endocrine neoplasia, type II (MEN II) is a disorder resulting in
tumors in more than one part of the body's hormone-producing (endocrine)
system. The locations of other tumors associated with MEN II include the
thyroid, parathyroid, lips, tongue and gastrointestinal tract.
•Von Hippel-Lindau disease can result in tumors at multiple sites, including
the central nervous system, endocrine system, pancreas and kidneys.
•Neurofibromatosis 1 (NF1) results in multiple tumors in the skin
(neurofibromas), pigmented skin spots and tumors of the optic nerve.
•Hereditary paraganglioma syndromes are inherited disorders that result in
either pheochromocytomas or paragangliomas.

7. "Five Hs"
Hypertension
Headache
Hyperhidrosis
Hypermetabolism
Hyperglycemia
Skin sensations
Flank pain Elevated heart rate
Palpitations
Anxiety often resembling that of a panic attack
Weight loss

8. • That causes palpitation
and increased-rapid heart
rate due to over
production of
catecholamines.
http://learn.genetics.utah.edu/content/cells/fight_flight/

9. The diagnosis can be established by measuring
catecholamines and metanephrines in plasma (blood) or
through a 24-hour urine collection
Laboratory tests (Blood Test)
One diagnostic test used in the past for a
pheochromocytoma is to administer clonidine, a
centrally-acting alpha-2 agonist used to treat high blood
pressure. Clonidine mimics catecholamines in the brain,
causing it to reduce the activity of the sympathetic
nerves controlling the adrenal medulla. A healthy adrenal
medulla will respond to the clonidine suppression test by
reducing catecholamine production; the lack of a
response is evidence of pheochromocytoma.

10. CT scans of large phaeochromocytoma (arrow, left & centre) and
paraganglioma (arrow, right)

11. MAGNETIC RESONANCE IMAGING

12. Malignant, metastatic
pheochromocytoma
demonstrated by 123-I-
Meta-iodobenzylguanidine
(MIBG)

13. Fluorodopamine Positron Emission
Tomographic (PET)

15. The doctor may order two
different drugs for a period of 7 to 10
days that help lower blood pressure
before surgery.
Alpha blockers prevent
noradrenaline from stimulating the
muscles in the walls of smaller arteries
and veins. Because these blood vessels
remain open and relaxed, blood flow
improves and blood pressure lessens.

16. Surgical resection of
the tumor is the treatment
of first choice, either by
open laparotomy or else
laparoscopy.
(ADRENALECTOMY
removal of one or both
adrenal glands)

17. Adrenalectomy a surgical removal of adrenal glands.

18. •Exercise Restrictions: If patient have been diagnosed
with a pheochromocytoma or paraganglioma, no
exercise until the lesion/tumor has been removed and the
catecholamines are down to a normal level. Exercise
increases catecholamine production.
• Instruct patient to avoid caffeine/decaf products, alcohol
and smoking.
•It is important that the patient get good nutrition and eat
a variety of healthy foods like fruits, vegetables, fish,
poultry, milk & cheese etc.

19. •Encourage liberal salt intake 1 month
before surgery.
• Check patient's blood sugar several times
each day. Blood pressure, heart rate, and
glucose levels should be always monitor.
•Encourage patient to learn ways to
manage stress. Deep breathing,
meditation, and listening to music may help
the patient to cope with stressful events.

20. • Decreased cardiac output related to
increased vascular resistance/
vasoconstriction
• Pain related to increased cerebral
vascular pressure as manifested by
headache
• Activity Intolerance related to body
weakness
• Knowledge deficit related to lack of
information about the disease process
and self-care

21. References:
Jospeh Bongon,1Raymond Oliva,2 Frances Lina Lantion-Ang., Terazosin
as First Line Preoperative Blockade in Filipino Patients Diagnosed with
Pheochromocytoma. Section of Endocrinology and Metabolism,
Department of Medicine, Philippine General Hospital.
Retrieved (July 19, 2015) from: http://www.asean-
endocrinejournal.org/index.php/JAFES/article/view/171/620
Diseases and conditions: Pheochromocytoma. Retrieved (July 19, 2015)
from: http://www.mayoclinic.org/diseases-
conditions/pheochromocytoma/basics/treatment/con-20030435.
William F Young, Jr, MD, MSc., Norman M Kaplan, MD., Clinical
presentation and diagnosis of pheochromocytoma. Retrieved (July 19,
2015) from: http://www.uptodate.com/contents/clinical-presentation-
and-diagnosis-of-
pheochromocytomahttp://www.pheochromocytoma.org/

22. Karel Pacak, M.D., Ph.D., D.Sc., Section on Medical
Neuroendocrinology, Pediatric and Reproductive Endocrinology
Branch, NICHD NIH, Bethesda, MD 20892,
USA endotext.org/adrenal/adrenal34/adrenal34.
Pheochromocytoma: Diagnosis, Localization, and Treatment By
Karel Pacak, Graeme Eisenhofer, Jacques W. M. Lenders
Plasma catecholamines and essential hypertension. DS Goldstein
- Hypertension, Vol 5, 86-99
Retrieved (July 19, 2015) from:
http://pheochromocytomasupportboard.yuku.com/topic/5645/P
heochromocytoma-symptoms-and-induced-
conditions#.Vff0S_noQ3x
Marilynn E. Doenges., Mary Frances Moorhouse., Alice C. Murr.,
Nurse’s Pocket Guide., Edition 13th., Diagnoses.