PHEOCHROMOCYTOMA.ppt

PHEOCHROMOCYTOMA Govind SRMCRI. FEATURES TUMOR FROM ADRENAL MEDULLA RARE TUMOR 0.1-1% OF HYPERTESIVES HAVE THIS TUMOR Arise from chromaffin cells RL Adrenal tumors secrete more of ephenephrine extra-adrenal tumors secrete norephinephrine. 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 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