PHEOCHROMOCYTOMA

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     1  PHEOCHROMOCYTOMA Govind SRMC&RI.
     2  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.
     3  PHENYLALANINE TYROSINE DOPA NOR EPINEPHRINE EPINEPHRINE HYDROXYLASE DECARBOXYLASE BETA HYDROXYLASE PNMT
     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 Malignancy…indicated 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 DD…eclampsia Usual time of presentation….post partum---labour Maternal and infant mortality…high(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  METANEPHRINE EPINEPHRINE 3,4 DIHYDROXYPHENYL GLYCOALDEHYDE 3METHOXY4HYDROXY PHENYLETHYLENE GLYCOL VMA NOREPINEPHRINE NORMETANEPHRINE COMT COMT MAO MAO METABOLISM OF CATACHOLAMINES
     13  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
     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 Prazocin……reversible 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 Avoid…cheese/ephdrine/succinylcholine/glucagon/nicotine/histamine/tyrosine Correct lactic acidosis
     17  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
     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 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
     22  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
     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 EVALUATE & REMOVE NONFUNCTIONAL < 5 cm SOLID MRI HIGH INTENSITY REMOVE CYSTIC FOLLOW UP REMOVE IF SIZE INCREASES OR IT BECOMES SYMPTOMATIC SOLID REMOVE > 5 cm
     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 > 5 cm to be removed CT tends to underestimate size…so 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 >3 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 < 6 cm and benign
     32  medical traetment…. Mitotane….DDT derivative 35% response 8-10g/day High toxicity rates