PHEOCHROMOCYTOMA

Report
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
 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

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
 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 actingirreversible binding)….start with 2030mg tid and increase up to 40100mg/day
 Prazocin……reversible
 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
 Avoid…cheese/ephdrine/succinylcholin
e/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
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 size…so
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


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