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
HYDROXYLASE
TYROSINE
DECARBOXYLASE
DOPA
BETA HYDROXYLASE
NOR EPINEPHRINE
PNMT
EPINEPHRINE

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 Tumors that produce

catocholamines
Somatostatin
Calcitonin
Oxytocin
vasopressin

ACTH

Chemodectoma
Ganglioneuroma
Ganglioneuroblasto
ma
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
Malignancyindicated 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
DDeclampsia
Usual time of presentation.post
partum---labour
Maternal and infant
mortalityhigh(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

METABOLISM OF CATACHOLAMINES
EPINEPHRINE

NOREPINEPHRINE
COMT

COMT
METANEPHRINE

NORMETANEPHRINE

MAO

MAO

3,4 DIHYDROXYPHENYL
GLYCOALDEHYDE

3METHOXY4HY
DROXY
PHENYLETHYL
ENE GLYCOL

VMA

ASSAY OF CATACHOLAMINES
URINE
Epinephrine
25mcg/d
NOREPI..75mcg/d
VMA..8mcg/d
Metanep..300mcg/d
Normeta450mcg/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 actingirreversible binding).start with 2030mg tid and increase up to 40100mg/day
Prazocinreversible
Metyrosine
(Alpha methyl paratyosine)250mg TID
Why first alpha blocker----then beta
blocker

PRE OP
Do echo cardiac pathology
Add beta blocker
Adequate hydration
Crystalloids use full
Avoidcheese/ephdrine/succinylcholin
e/glucagon/nicotine/histamine/tyrosine
Correct lactic acidosis

INTRA OP
Intraopproblems 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 produceinactive
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 peaks4th decade & <6yrs
Lesions > 6 cm to be considered
malignant

Incidentaloma0.61.3% 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

NONFUNCTIONAL

EVALUATE & REMOVE

> 5 cm

< 5 cm
SOLID

CYSTIC

MRI

FOLLOW
UP

HIGH INTENSITY

REMOVE

REMOVE IF
SIZE
INCREASES OR
IT BECOMES
SYMPTOMATIC

SOLID

REMOVE

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 sizeso
5cm ..

FUNCTIONAL TUMORS
ADENAL CARCINOMA
ADRENAL CARCINOMA
WITH CUSHINGS
WITH HYPERALDO
PURE/MIXED
SIZE OF TUMOR IS
USUALLY >3 CM
(VIRILIZATION)
COMMONLY
PURE FORM IS
ASSOCIATED
LESS COMMON
WITHCORTISOL OR
ANDROGEN EXCESS
17KETOSTEROIDS
ADRENAL
& DHEA ARE
ADENOMA/CAH HAVE TO
ELEVATED
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