PHEOCHROMOCYTOMA - PowerPoint PPT Presentation

View by Category
About This Presentation
Title:

PHEOCHROMOCYTOMA

Description:

PHEOCHROMOCYTOMA Govind SRMC&RI. FEATURES TUMOR FROM ADRENAL MEDULLA RARE TUMOR 0.1-1% OF HYPERTESIVES HAVE THIS TUMOR Arise from chromaffin cells RL Adrenal tumors ... – PowerPoint PPT presentation

Number of Views:805
Avg rating:3.0/5.0
Date added: 18 May 2020
Slides: 33
Provided by: medindiaN4
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

Title: PHEOCHROMOCYTOMA


1
PHEOCHROMOCYTOMA
  • Govind
  • SRMCRI.

2
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.

3
PHENYLALANINE
HYDROXYLASE
TYROSINE
DECARBOXYLASE
DOPA
BETA HYDROXYLASE
NOR EPINEPHRINE
PNMT
EPINEPHRINE
4
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

5
  • Abnormal secretions
  • Somatostatin
  • Calcitonin
  • Oxytocin
  • vasopressin
  • ACTH
  • Tumors that produce catocholamines
  • Chemodectoma
  • Ganglioneuroma
  • Ganglioneuroblastoma
  • neuroblastoma

6
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

7
10 TUMOR
  • 10 extra adrenal
  • 10 malignant
  • 10 familial
  • 1 - neck / thorax / bladder
  • 10 bilateral

8
ASSOCIATION
  • TSC
  • Von recklinghausens disease
  • MEN 2a 2b
  • 2a-PH,PTA,MCT,RCC
  • 2b NO RCC
  • Von hippel
  • Struge weber

9
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

10
PHEO PREGNENCY
  • Present with HT/headache/palpitations
  • DDeclampsia
  • Usual time of presentation.post partum---labour
  • Maternal and infant mortalityhigh(40)

11
PHEO HEART
  • Catcholamine induced cardiomyopathy
  • Myocardial inflamation/fibrosis
  • Poor myocardial pump function
  • Decrease in viable myofibrils
  • All patients need ECG/ECHO/ISOTOPLE HEART SCAN

12
METABOLISM OF CATACHOLAMINES
NOREPINEPHRINE
EPINEPHRINE
COMT
COMT
METANEPHRINE
NORMETANEPHRINE
MAO
MAO
3,4 DIHYDROXYPHENYL GLYCOALDEHYDE
3METHOXY4HYDROXY PHENYLETHYLENE GLYCOL
VMA
13
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

14
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)

15
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

16
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

17
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

18
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

19
FOLLOW UP
  • Urine catacholamines
  • Serum levels
  • CT / MRI
  • Bone scan
  • MIBG

20
ADRENAL CARCINOMA
21
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

22
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

23
CLASSIFICATION
  • NON FUNCTIONAL
  • FUNCTIONAL
  • CUSHINGS
  • VIRULISING
  • FEMINISING
  • HYPERALDO
  • MIXED

24
STAGING
  • STAGE I T1 N0M0
  • STAGE II T1 N0M0
  • STAGE III T3N0M0
  • T1/T2 NIM0
  • STAGE IV T4
  • T3 NIMO
  • any T with M1

25
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.

26
MODIFIED PROTOCOL
CT AND OR MRI
R/O PHEO URINE CATACHOLAMINES MRI
SERUM GLUCOCORTICOID LEVELS WITH OR WITHOUT SEX
HORMONE
27
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
28
METASTATIC TUMORS
  • Melanoma
  • Breast CA
  • Lung CA
  • RCC.upto 40

29
  • 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 ..

30
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

31
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

32
medical traetment.
  • Mitotane.DDT derivative
  • 35 response
  • 8-10g/day
  • High toxicity rates
About PowerShow.com