Adrenal Medulla Pheochromocytoma

DEFINITIONDEFINITION

Pheochromocytoma is a tumor of Pheochromocytoma is a tumor of special cells (called chromaffin special cells (called chromaffin cells), most often found in the cells), most often found in the adrenal medula.adrenal medula.

CAUSESCAUSES • Pregnancy Pregnancy • Lifting Lifting • Urination Urination • DrugDrug• Withdrawal from drug Withdrawal from drug • Panic attacksPanic attacks• Spinal cord injuriesSpinal cord injuries

RISK GROUPRISK GROUP• Equal occurrence in females and malesEqual occurrence in females and males• Can occur at any age. Most common in people Can occur at any age. Most common in people

between 30 and 60between 30 and 60• Usually on Right sideUsually on Right side

• Incidence decreases with age Incidence decreases with age

• headaches headaches • excess sweating excess sweating • racing heart racing heart • rapid breathing rapid breathing • anxiety/nervousness anxiety/nervousness • nervous shaking nervous shaking • pain in the lower chest or upper abdomen pain in the lower chest or upper abdomen • nausea nausea • heat intoleranceheat intolerance

Clinical manifestation

PHEOCHROMOCYTOMA IN CHILDREN PHEOCHROMOCYTOMA IN CHILDREN AND PREGNANCY AND PREGNANCY

PHEOCHROMOCYTOMA IN CHILDRENPHEOCHROMOCYTOMA IN CHILDREN

• Pheochromocytoma is rare in children, but occurs most commonly Pheochromocytoma is rare in children, but occurs most commonly between the ages of 8 and 14 years. Diagnosis of pheochromocytoma between the ages of 8 and 14 years. Diagnosis of pheochromocytoma can be more difficult at this age, because other can be more difficult at this age, because other childhood cancerschildhood cancers (e.g. (e.g. neuroblastomaneuroblastoma) can also elevate adrenaline compounds in the body. ) can also elevate adrenaline compounds in the body.

• Pheochromocytomas in children are more likely to be Pheochromocytomas in children are more likely to be bilateralbilateral (on both (on both the left and right sides of the body) and outside the adrenal glands. For the left and right sides of the body) and outside the adrenal glands. For this reason, transabdominal surgery is usually performed to remove the this reason, transabdominal surgery is usually performed to remove the tumor.tumor.

PHEOCHROMOCYTOMA IN PHEOCHROMOCYTOMA IN PREGNANCYPREGNANCY• Although rare, pheochromocytoma in pregnancy Although rare, pheochromocytoma in pregnancy

can be very dangerous. Because x rays are to be can be very dangerous. Because x rays are to be avoided in pregnancy, MRI and/or ultrasound is avoided in pregnancy, MRI and/or ultrasound is used to locate the tumor. Alpha-adrenergic used to locate the tumor. Alpha-adrenergic blocking agents to reduce blood pressure are blocking agents to reduce blood pressure are given to the woman as soon as the diagnosis is given to the woman as soon as the diagnosis is made. made.

• If the woman is in the first two trimesters of If the woman is in the first two trimesters of pregnancy, most often the tumor is removed. In pregnancy, most often the tumor is removed. In the third trimester, the woman usually remains on the third trimester, the woman usually remains on alpha-adrenergic blocking agents until a cesarean alpha-adrenergic blocking agents until a cesarean section can be safely performed.section can be safely performed.

INVESTIGATIONINVESTIGATION• Genetic testingGenetic testing

• CT scan or MRICT scan or MRI

• 24 hours urine collection24 hours urine collection

• AngiographyAngiography

• Radioactive iodineRadioactive iodine

TREATMENTTREATMENT• Beta blockersBeta blockers

• IV phentolamineIV phentolamine

• Radiation therapyRadiation therapy

• ChemotherapyChemotherapy

• Alpha blockersAlpha blockers

• PhenoxybenzaminePhenoxybenzamine

• labetalol (to slow the heart rate)labetalol (to slow the heart rate)

• atenololatenolol

• reninangiotensinreninangiotensin

• catecholaminescatecholamines

SURGICAL TREATMENTSURGICAL TREATMENT

• AdrenalectomyAdrenalectomy

• LaparotomyLaparotomy

• LaparoscopyLaparoscopy

• Transabdominal surgeryTransabdominal surgery

CT ABDOMEN

COMPLICATIONCOMPLICATION

• Heart failure Heart failure • Stroke Stroke • Kidney failure Kidney failure • Acute respiratory distress Acute respiratory distress • Confusion Confusion • Psychosis Psychosis • Seizures Seizures • Visual impairment Visual impairment • Premature death Premature death

CONT…CONT…Long-term exposure to these Long-term exposure to these

hormones canhormones can

cause:cause:

• Damage to your heart muscle Damage to your heart muscle • Congestive heart failure Congestive heart failure • An increased risk of diabetes An increased risk of diabetes

PRE-OPERATIVE CAREPRE-OPERATIVE CARE

• Request a dietary consultation to Request a dietary consultation to discuss with client about a diet discuss with client about a diet high in vitamin and protein.high in vitamin and protein.

• Use careful medical and surgical Use careful medical and surgical asepsis when providing care and asepsis when providing care and treatments.treatments.

• Monitor the results of laboratory Monitor the results of laboratory test of electrolytes and glucose test of electrolytes and glucose level.level.

• Teach the client to turn, cough Teach the client to turn, cough and perform deep-breathing and perform deep-breathing exercise.exercise.

• Frequently observe whereby the Frequently observe whereby the blood pressure and fluid are blood pressure and fluid are optimizedoptimized

• Surgery and nursing care are Surgery and nursing care are explained to patient.explained to patient.

• Prepare patient for for major Prepare patient for for major abdominal surgeryabdominal surgery

POST-OPERATIVE CAREPOST-OPERATIVE CARE

• Record vital sign, measure intake and Record vital sign, measure intake and output, and monitor electrolytes on a output, and monitor electrolytes on a frequent schedule, especially during frequent schedule, especially during the 48 hour after surgery.the 48 hour after surgery.

• Assess body temperature, WBC level Assess body temperature, WBC level and wound drainage, change and wound drainage, change dressing using sterile techniquedressing using sterile technique

• Encourage early ambulation; slow Encourage early ambulation; slow progression of diet when bowel progression of diet when bowel sound return.sound return.

• Administer iv hydrocortisone is given Administer iv hydrocortisone is given as directed to prevent adrenal crisisas directed to prevent adrenal crisis

• Monitor serum sodium, potassium Monitor serum sodium, potassium and glucose for any abnormalities.and glucose for any abnormalities.

• Assess dressing for leakage Assess dressing for leakage internally, and after it has been internally, and after it has been changed, assess wound for sign for changed, assess wound for sign for infection.infection.

High magnificant micrograph of a pheochromocytoma showing the nested arrangement of cells(Zellballen)and stippled chromatin.

Histopathology of adrenal pheochromocytoma. Adrenectomy specimen

Micrograph of pheochromocytoma

Bilateral of pheochromocytoma

Group members…Group members…

DarmaDeviDarmaDeviPrashalahPrashalahLetchumiLetchumiSuhanimahSuhanimahThevikaThevikaAdilah AmniAdilah AmniNurshuhadaNurshuhadaNuraisyahNuraisyahHajar NursyamimiHajar NursyamimiNurasyikin Nurasyikin