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