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PHEOCHROMOCYTOMA

Lt Col Syeda Rifaat Naqvi


Consultant Breast and Colorectal
Surgeon
FEATURES
 TUMOR FROM ADRENAL MEDULLA
 RARE TUMOR
 0.1-1% OF HYPERTESIVES HAVE
THIS TUMOR
 Arise from chromaffin cells
 R>L
 Adrenal tumors secrete more of
ephenephrine & extra-adrenal tumors
secrete norephinephrine.
PRESENTATION
 Sustained HT ( children & MEN 2)
 Paroxysms of HT (women)
 Sustained with paroxysms
 Headache/vomiting/visual disturbance
 hypoglycemia
 Palpitations/CVA/COMA
 Sweating/pallor/flushing/chest pain
10% TUMOR

 10% extra adrenal


 10% malignant
 10% familial
 10 % bilateral
ASSOCIATION

 Von
Recklinghausen
s disease
 MEN 2A & 2B
 Von hippel
Lindau Disease
 Struge weber
METABOLISM OF CATACHOLAMINES

NOREPINEPHRINE
EPINEPHRINE

COMT COMT

METANEPHRINE NORMETANEPHRINE
MAO MAO
3,4 DIHYDROXYPHENYL
GLYCOALDEHYDE

3METHOXY4HY
DROXY VMA
PHENYLETHYL
ENE GLYCOL
ASSAY OF CATECHOLAMINES

URINE BLOOD
 Epinephrine  EPINEP….
25mcg/d 15-50pg/ml
 NOREPI..75mcg/d  NOREPI…
 VMA…..8mcg/d 50-500pg/ml
 Metanep..300mcg/d  Dopamine…
 Normeta…450mcg/d <100pg/ml
INVESTIGATIONS

 CT homogenous enhancing lesion


 MRI T2(3 times brighter than liver)
LIGHT BULB
 MIBG SCAN – extra adrenal lesions
 PET with 2-flourodeoxy D glucose
 Bone scan
MANAGEMENT OF HT
 Alpha blocker----then beta blocker
INTRA OP
 Intraop…problems at time of
….induction and handling of tumor
 Have….ECG,CVP,PCWP,output
monitoring.
 Have at hand..alpha & beta blockers
loaded IV at hand
 Phentolamine 50mg in 500ml NS
 Sodium nitroprusside 50 mg in 250ml
5% dextrose
POST OP
 Post op 75% have normal BP and rest
25% have easily controllable BP
 Urine catacholamines return to normal
in 1 week
 Tumor recurrence seen in 10%
 Bony mets..best is bone scan
 Follow up
FOLLOW UP

 Urine catecholamines
 Serum levels
 CT / MRI
 Bone scan
 MIBG

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