pheochromocytoma govind srmc&ri.. features tumor from adrenal medulla rare tumor 0.1-1% of...
TRANSCRIPT
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PHEOCHROMOCYTOMA
Govind
SRMC&RI.
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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.
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PHENYLALANINE
TYROSINE
DOPA
NOR EPINEPHRINE
EPINEPHRINE
HYDROXYLASE
DECARBOXYLASE
BETA HYDROXYLASE
PNMT
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PRESENTATION
Sustained HT ( children & MEN 2) Paroxysms of HT (women) Sustained with paroxysms Headache/vomiting/visual disturbance hypoglycemia Polyuria/polydipsia Palpitations/CVA/COMA Sweating/pallor/flushing/chest pain
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Abnormal secretions
Somatostatin Calcitonin Oxytocin vasopressin ACTH
Tumors that produce catocholamines
Chemodectoma
Ganglioneuroma
Ganglioneuroblastoma
neuroblastoma
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FEATURES
Some cases (upto 10%) need not have HT
Frequency of paroxysm Polyuria,polydipsia rare in adults but
seen in 25% children 95% cases sporadic Malignancy…indicated by…
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10% TUMOR
10% extra adrenal 10% malignant 10% familial 1% - neck / thorax / bladder 10 % bilateral
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ASSOCIATION
TSC Von recklinghausens disease MEN 2a & 2b
2a-PH,PTA,MCT,RCC
2b – NO RCC Von hippel Struge weber
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PHEO IN CHILDREN Headache/nausea/vomiting Wt loss
common 15-30 % multiple 24 % B/L 10% familial 15-30 extra adrenal HT is sustained Malignancy more common Polyuria/polydipsia/convulsions 25%
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PHEO & PREGNENCY
Present with HT/headache/palpitations DD…eclampsia Usual time of presentation….post
partum---labour Maternal and infant mortality…
high(40%)
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PHEO & HEART
Catcholamine induced cardiomyopathy Myocardial inflamation/fibrosis Poor myocardial pump function Decrease in viable myofibrils All patients need
ECG/ECHO/ISOTOPLE HEART SCAN
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METANEPHRINE
EPINEPHRINE
3,4 DIHYDROXYPHENYL
GLYCOALDEHYDE
3METHOXY4HYDROXY PHENYLETHYLENE GLYCOL
VMA
NOREPINEPHRINE
NORMETANEPHRINE
COMT COMT
MAO MAO
METABOLISM OF CATACHOLAMINES
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ASSAY OF CATACHOLAMINES
URINE Epinephrine
25mcg/d NOREPI..75mcg/d VMA…..8mcg/d Metanep..300mcg/d Normeta…450mcg/d
BLOOD EPINEP….
15-50pg/ml NOREPI…
50-500pg/ml Dopamine…
<100pg/ml
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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 Clonodine suppression test…..(300mcg)
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MANAGEMENT OF HT
Phenoxybenzamine ( long acting-irreversible binding)….start with 20-30mg tid and increase up to 40-100mg/day
Prazocin……reversible Metyrosine(Alpha methyl paratyosine)…250mg TIDWhy first alpha blocker----then beta
blocker
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PRE OP
Do echo ……cardiac pathology Add beta blocker Adequate hydration Crystalloids use full Avoid…cheese/ephdrine/succinylcholine/
glucagon/nicotine/histamine/tyrosine Correct lactic acidosis
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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
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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…….since of the cases who
recur 5% every year occur
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FOLLOW UP
Urine catacholamines Serum levels CT / MRI Bone scan MIBG
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ADRENAL CARCINOMA
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INTRODUCTION Most adrenal malignant tumors are
functional Nonfunctional can become functional
over a period of time. Some tumors produce……inactive
metabolites or very little amount of substances that even though they are active they are clinically nonfunctional
Very rae to be detected at autopsy
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FEATURES
Tumor of the cortex F:M – 2:1 R>L Age….two peaks…4th decade & <6yrs Lesions > 6 cm to be considered
malignant Incidentaloma……0.6—1.3% of CT ABD
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CLASSIFICATION
NON FUNCTIONAL FUNCTIONAL
CUSHINGS
VIRULISING
FEMINISING
HYPERALDO
MIXED
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STAGING
STAGE I : T1 N0M0 STAGE II : T1 N0M0 STAGE III : T3N0M0
T1/T2 NIM0 STAGE IV : T4
T3 NIMO
any T with M1
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INVESTIGATION
CT homogenous lesion MRI T2 images bright lesion (as bright
as liver)DD neural tumors/metastatic/hemorrhage
FNAC….no material 30% But if material is suffuciant….diagnostic
accuracy is 95%.
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MODIFIED PROTOCOL
CT AND OR MRI
R/O PHEO
URINE CATACHOLAMINES & MRI
SERUM GLUCOCORTICOID LEVELS
WITH OR WITHOUT SEX HORMONE
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ADRENAL MASS FUNCTIONAL
EVALUATE & REMOVENONFUNCTIONAL
< 5 cm
SOLID
MRI
HIGH INTENSITY
REMOVE
CYSTIC
FOLLOW UP
REMOVE IF SIZE INCREASES OR IT BECOMES SYMPTOMATIC
SOLID
REMOVE
> 5 cm
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METASTATIC TUMORS
Melanoma Breast CA Lung CA
RCC….upto 40%
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Adenoma are smaller Usually functional Difficult to differentiate from
malignancy by HPE Tumors reported initially as adenoma ,
later on have had mets This is why all tumors > 5 cm to be
removed CT tends to underestimate size…so
5cm ..
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FUNCTIONAL TUMORS
ADENAL CARCINOMA WITH CUSHINGS
PURE/MIXED (VIRILIZATION)
PURE FORM IS LESS COMMON
17KETOSTEROIDS & DHEA ARE ELEVATED
ADRENAL CARCINOMA WITH HYPERALDO
SIZE OF TUMOR IS USUALLY >3 CM
COMMONLY ASSOCIATED WITHCORTISOL OR ANDROGEN EXCESS
ADRENAL ADENOMA/CAH HAVE TO BE RULED OUT
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FUNCTIONAL TUMORS
FEMINISING TUMOR Men 25-50yrs Large/palpable Highly malignant(80) Gynacomastia Testicular
atropy/impotance Tumor androstenidione
is converted peripherally into estrogen
VIRILIZING TUMOR Usually associated with
cushings Pure form is more often
due to ovarian tumor Adrenal tumor may
have ledig cell adenoma/nodule
Usually size < 6 cm and benign
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medical traetment….
Mitotane….DDT derivative 35% response 8-10g/day High toxicity rates