PHEOCHROMOCYTOMA

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PHEOCHROMOCYTOMA

• Govind
• SRMCRI.

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FEATURES

• TUMOR FROM ADRENAL MEDULLA
• RARE TUMOR
• 0.1-1 OF HYPERTESIVES HAVE THIS TUMOR
• Arise from chromaffin cells
• RgtL
• Adrenal tumors secrete more of ephenephrine
  extra-adrenal tumors secrete norephinephrine.

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PHENYLALANINE
HYDROXYLASE
TYROSINE
DECARBOXYLASE
DOPA
BETA HYDROXYLASE
NOR EPINEPHRINE
PNMT
EPINEPHRINE
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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
• Malignancyindicated 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
• DDeclampsia
• Usual time of presentation.post partum---labour
• Maternal and infant mortalityhigh(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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METABOLISM OF CATACHOLAMINES
NOREPINEPHRINE
EPINEPHRINE
COMT
COMT
METANEPHRINE
NORMETANEPHRINE
MAO
MAO
3,4 DIHYDROXYPHENYL GLYCOALDEHYDE
3METHOXY4HYDROXY PHENYLETHYLENE GLYCOL
VMA
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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
• lt100pg/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
• Prazocinreversible
• Metyrosine
• (Alpha methyl paratyosine)250mg TID
• Why first alpha blocker----then beta blocker

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PRE OP

• Do echo cardiac pathology
• Add beta blocker
• Adequate hydration
• Crystalloids use full
• Avoidcheese/ephdrine/succinylcholine/glucagon/nic
  otine/histamine/tyrosine
• Correct lactic acidosis

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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

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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 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

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FEATURES

• Tumor of the cortex
• FM 21
• RgtL
• Age.two peaks4th decade lt6yrs
• Lesions gt 6 cm to be considered malignant
• Incidentaloma0.61.3 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
NONFUNCTIONAL
EVALUATE REMOVE
gt 5 cm
lt 5 cm
SOLID
SOLID
CYSTIC
MRI
FOLLOW UP
REMOVE
HIGH INTENSITY
REMOVE IF SIZE INCREASES OR IT BECOMES SYMPTOMATIC
REMOVE
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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 gt 5 cm to be removed
• CT tends to underestimate sizeso 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 gt3 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 lt 6 cm and benign

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medical traetment.

• Mitotane.DDT derivative
• 35 response
• 8-10g/day
• High toxicity rates