Pheochromocytoma is a rare tumor that develops from chromaffin cells in the adrenal medulla or extra-adrenally. It secretes excess catecholamines (norepinephrine, epinephrine, dopamine). Most tumors (80-90%) develop in the adrenal medulla. Diagnosis involves measuring plasma and urine catecholamines. Treatment includes pharmacological therapy with alpha-adrenergic blockers before surgery and surgical removal of the tumor. Post-operatively, patients require monitoring of blood pressure, glucose, fluid balance and symptoms due to potential persistent or recurrent hypertension.
Pheochromocytoma is a rare tumor that develops from chromaffin cells in the adrenal medulla or extra-adrenally. It secretes excess catecholamines (norepinephrine, epinephrine, dopamine). Most tumors (80-90%) develop in the adrenal medulla. Diagnosis involves measuring plasma and urine catecholamines. Treatment includes pharmacological therapy with alpha-adrenergic blockers before surgery and surgical removal of the tumor. Post-operatively, patients require monitoring of blood pressure, glucose, fluid balance and symptoms due to potential persistent or recurrent hypertension.
Pheochromocytoma is a rare tumor that develops from chromaffin cells in the adrenal medulla or extra-adrenally. It secretes excess catecholamines (norepinephrine, epinephrine, dopamine). Most tumors (80-90%) develop in the adrenal medulla. Diagnosis involves measuring plasma and urine catecholamines. Treatment includes pharmacological therapy with alpha-adrenergic blockers before surgery and surgical removal of the tumor. Post-operatively, patients require monitoring of blood pressure, glucose, fluid balance and symptoms due to potential persistent or recurrent hypertension.
Pheochromocytoma is a rare tumor that develops from chromaffin cells in the adrenal medulla or extra-adrenally. It secretes excess catecholamines (norepinephrine, epinephrine, dopamine). Most tumors (80-90%) develop in the adrenal medulla. Diagnosis involves measuring plasma and urine catecholamines. Treatment includes pharmacological therapy with alpha-adrenergic blockers before surgery and surgical removal of the tumor. Post-operatively, patients require monitoring of blood pressure, glucose, fluid balance and symptoms due to potential persistent or recurrent hypertension.
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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
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
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
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 #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
