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PHEOCHROMOCYTOMA

Yosi Oktarina, S.Kep., Ners., M.Kep

ANATOMI

Pheochromocytoma
Tumors derives from chromafiin cells in the

adrenal medulla, or extra adrenal (para


ganglioma) medulla that secretes execessive
cathecolamine
Mostly beningn
Chromaffin Cells are found in the adrenal
medulla and the ganglia of the sympathetic
nervous system.

Contd
it releases norephinephrine, epinephrine,

dopamine
80-90% tumors develop in medulla adrenal
sometimes it depelovs in extra adrenal
(paraganglia)

Extra-Adrenal Sites
Within the sympathetic
nerve chain along the
spinal cord orange
spots ()
Overlying the distal
aorta (the main artery
from the heart) (green
spots)
Within the ureter
(collecting system from
the kidney(yellow
spot)
Within the urinary
bladder(blue spot)
Remember, 90% are in

Norepinephrine stimulate alpha receptor, but


it can also stimulate beta receptor
Epinephrine stimulate beta receptor
receptor
Found on smooth muscle
tissue
positive inotropic effects
vasocontrictor

receptor
Vasodilation (
cardioacceleration
increasing contractility
bronchodilation
Lypolisis
Glycognolysis

Patients with tumors that produces high


concentrations of norephinephrine are
likely to incur sustained hypertension, while
patients with significanly elevated levels of
epinephrine
are
often
seen
having
paroxymal hypertension. Patient with
dopamine secreting tumors are most often
normotensive (Proye, et al., 1986; Ito, Y., et
al., 1992)

Factors Associated with


Pheochromocytoma
Family history of pheochromocytoma
Genetic diseases :

- Van Hippel Lindau Disease


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.

Contd
- Multiple endocrine neoplasia, type 2 :
Two endocrine neoplasia tumor types
occur in the body with evidence of either
a causative mutation or hereditary
transmission.
- Neurofibromatosis type 1

Signs and Symptoms


The classical triad :
Pain (headache)
Perspiration
Palpitation

Signs and Symptoms

Hypertension (80-90%)
Hyperglicemia
Tachicardia
Anxiety
Chest pain
Palpitation
Severe headache
Flushing
Diaphoresis
Abdominal pain
Increased appetite
Weight loss

Diagnostic Test
MRI: identify location of tumor
Plasma level of cathecolamine : (norephinephri

and ephinephrine)
Glucose Test
Adrenal Biopsy
: identify feocromocytoma
Abdominal CT Scan : identify location of tumor
Clonidine supression : unlike normals, the
cathecolamine plasma will not decrease on
pheochromocytoma
24 hr urine sample : increase the excretion of
cathecolamine

TREATEMENT
Pharmacologic Therapy :
Decrease BP

- Alpha adrenergic blocking agents : Phentolamine


(Regitine)
- Smooth muscle relaxants : Na nitroprussid
(Nipridel)
Before and during surgery

- Beta adrenergic blocker : propanolol


- Alpha adrenergic blocker : Fenoksibenzamin
- Synthesis cathecolamine blocker : metyrosine

Surgical Therapy :

- adrenalectomy
- Hydration : IV fluid
- nipride
- alpha adrenergic blocker
- IV corticosteroid replacement : solumedrol
- Oral corticosteroid : prednison

POST OP MANAGEMENT
ECG
Arterial pressure
Fluid and electrolyte balance
Level of glucose

40% patients continue to be hypertensive :

- Not all tissue removed


- Reccurence
- Blood vessel damage by severe &
prolongod hypertension
Release of homon norephinephrine and
epinephrine returns to normal
Tumorreccurence seen in 10%

NURSING DIAGNOSIS
1.

Decrease cardiac output related to altered


stroke volume (vasocnstriction) evidence by
heart palpitation, tachychardi, ECG change
Outcomes : Increase of Cardiac Output

Intervention :
Monitor vital signs frequently
Monitor cardiac, rhythm and rate
Auscultate heart sound
Monitor ECG

NURSING ASSESMENT
The Five Hs
Hypertension
Headache
Hyperhidrosis
Hypermetabolism
Hyperglycemia

Blood sugar

- Hypoglycemia (after surgery)


- hyperglycemia (before and during
surgery)
Blood pressure
- Hypertension (before and during surgery)
- Hypotension (after surgery)
Vital sign
Haemodynamic parameter
Fluid and electrolyte status

Monitor fluid balance


Limit enviromental stimuli
Administer medication
Monitor effectiveness of medication

2. Ineffective peripheral tissue perfusion related


to hypertension evidenced by decrease in
peripheral pulses, CRT > 3, alteration in skin
characteristic
Outcomes : Peripheal tissue perfusion effective
Intervension :
Monitor Vital signs frequently
Administered oxygen therapy
Administered iv fluid

Monitor fluid status


Encourage of active or passive ROM
Monitor skin color and temperature
Evaluate peripheral pulses

Question 1
1. 90% pheochromocytoma banyak

ditemukan di :
a. Sepanjang saraf simpatis
b. medula adrenal
c. DI dalam kandung kemih
d. Semua jawaban di atas benar

Question 2
Pheochromocytoma mengganggu
pengeluaran hormon?
1.TSH
2.FSH
3.EPHINEPHRIN DAN NOREPINEPHRINE
4.ACTH

QUESTION #3
Di bawah ini manakah gejala yang paling
umum ditemukan pada pasien dengan
pheochromocytoma?
1.Pyuresis
2.Mual dan muntah
3.Ansietas
4.hipertensi

QUESTION #4
Penegakkan diagnosis dari
pheochromocytoma dapat menggunakan
1.Urine 24 jam
2. MRI
3. Clonidin supresi
4. Kadar glukosa darah

QUESTION #5
Masalah keperawatan yang mungkin
diangkat pada pasien dengan
pheochromocytoma adalah..
1.Penurunan curah jantung
2.Ansietas
3.Perfusi jaringan tidak efektif
4.Gangguan pertukaran gas