pheochromocytoma govind srmc&ri.. features tumor from adrenal medulla rare tumor 0.1-1% of...
TRANSCRIPT
PHEOCHROMOCYTOMA
Govind
SRMC&RI.
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.
PHENYLALANINE
TYROSINE
DOPA
NOR EPINEPHRINE
EPINEPHRINE
HYDROXYLASE
DECARBOXYLASE
BETA HYDROXYLASE
PNMT
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
Abnormal secretions
Somatostatin Calcitonin Oxytocin vasopressin ACTH
Tumors that produce catocholamines
Chemodectoma
Ganglioneuroma
Ganglioneuroblastoma
neuroblastoma
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…
10% TUMOR
10% extra adrenal 10% malignant 10% familial 1% - neck / thorax / bladder 10 % bilateral
ASSOCIATION
TSC Von recklinghausens disease MEN 2a & 2b
2a-PH,PTA,MCT,RCC
2b – NO RCC Von hippel Struge weber
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%
PHEO & PREGNENCY
Present with HT/headache/palpitations DD…eclampsia Usual time of presentation….post
partum---labour Maternal and infant mortality…
high(40%)
PHEO & HEART
Catcholamine induced cardiomyopathy Myocardial inflamation/fibrosis Poor myocardial pump function Decrease in viable myofibrils All patients need
ECG/ECHO/ISOTOPLE HEART SCAN
METANEPHRINE
EPINEPHRINE
3,4 DIHYDROXYPHENYL
GLYCOALDEHYDE
3METHOXY4HYDROXY PHENYLETHYLENE GLYCOL
VMA
NOREPINEPHRINE
NORMETANEPHRINE
COMT COMT
MAO MAO
METABOLISM OF CATACHOLAMINES
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
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)
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
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
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…….since of the cases who
recur 5% every year occur
FOLLOW UP
Urine catacholamines Serum levels CT / MRI Bone scan MIBG
ADRENAL CARCINOMA
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
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
CLASSIFICATION
NON FUNCTIONAL FUNCTIONAL
CUSHINGS
VIRULISING
FEMINISING
HYPERALDO
MIXED
STAGING
STAGE I : T1 N0M0 STAGE II : T1 N0M0 STAGE III : T3N0M0
T1/T2 NIM0 STAGE IV : T4
T3 NIMO
any T with M1
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%.
MODIFIED PROTOCOL
CT AND OR MRI
R/O PHEO
URINE CATACHOLAMINES & MRI
SERUM GLUCOCORTICOID LEVELS
WITH OR WITHOUT SEX HORMONE
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
METASTATIC TUMORS
Melanoma Breast CA Lung CA
RCC….upto 40%
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 ..
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
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
medical traetment….
Mitotane….DDT derivative 35% response 8-10g/day High toxicity rates