adrenal tumors ppt.pptx presentation....

Adrenal tumors

1. ADRENAL TUMORS

2. ANATOMY Paired glands , weighing 4g Retroperitoneal Near upper poles of kidneys , within gerotas facial Right adrenal gland – between right love of liver and diaphragm, partly behind IVC Left – superomedial to upper poles of left kidney , covered by pancreatic tail and spleen

3. BLOOD SUPPLY Multiple small arterial branches from The aorta Inferior phrenic artery Renal artery Venous drainage Right – IVC Left – Renal vein

4. PHYSIOLOGY Zona glomerulosa – Aldosterone Zona fasciculata – cortisol regulated by ACTH Zona reticularis – Androgens – DHEA – under control of ACTH

5. INCIDENTALOMA An asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease Etiology Benign and malignant tumors of cortex , medulla , extra adrenal origin Functional or non functional – determined through clinical assessment Dexamethasone suppression test measurement of urinary metanephrins Plasma Aldosterone renin ratio 2% incidence

6. Benign or Malignant Clinically cushings and virilising tumors – Malignant Non contrast CT – Benign tumors <10HU PET /MRI

7. INVESTIGATION AND MANAGEMENT ALGORITHM FOR INCIDENTALOMA

8. CORTICAL TUMORS ALDOSTERONE PRODUCING ADENOMA( Primary hyperaldosteronism ) Arises from Zona glomerulosa Max diameter 10 to 20mm Macroscopically – Well circumscribed , golden yellow in color ( canary tumor ) Mutations in potassium coded channels , triggering calcium influx into glomerulosa cells C/F HTN Hypokalemia Can be asymptomatic

9. Diagnosis Non suppressed plasma Aldosterone and suppressed plasma renin activity Radiologically – CT and PET Treatment Prior to surgery – HTN and hypokalemia to be corrected Surgery – Adrenalectomy

10. ADRENAL ADENOMA Most common cause of adrenal Cushings syndrome Well circumscribed, modular in appearance Results in hypercortisolim leading to suppressed ACTH , causing atrophy of fasciculata , reticularis not glomerulosa

11. DIAGNOSIS Late night salivary cortisol levels 24 hr urinary free cortisol excretion - >3 times of upper limit Non suppression of morning cortisol levels after overnight dexamethasone suppression test Radiologically – Adrenal CT or MRI

12. DEXAMETHASONE SUPPRESSION TEST In this test, 1 mg of a synthetic glucocorticoid (dexamethasone) is given at 11 p.m. and plasma cortisol levels are measured at 8 a.m. the following morning . Physiologically normal adults suppress cortisol levels to <3 μ g/dL, whereas most patients with Cushing’s syndrome do not. In patients with a negative test but a high clinical suspicion, the classic low-dose dexamethasone (0.5 mg every 6 hours for eight doses, or 2 mg over 48 hours) suppression test or urinary cortisol measurement should be performed.

13. TREATMENT Unilateral adenoma – Adrenalectomy Bilateral Adrenalectomy – in severe and symmetrically enlarged adrenal Asymmetric disease – excision of larger gland

14. ADRENOCORTICAL CARCINOMA Rare aggressive malignancy from adrenal cortex Presents at late stage with poor prognosis Sporadic – majority , or as a part of tumor syndromes ( MEN 1 ) Incidence – 1 in 1 lakh Female preponderance Non functional > functional

15. Clinical features Abdominal lump , Abdominal or back pain Features of Cushing syndrome Pathology Large tumors with necrosis Capsular invasion Abnormal mitosis Investigations MRI CT 24 hr uninary metanephrins Overnight dexamethasone suppression test

16. Treatment Indeterminate or probably malignant tumor < 6cm : laparoscopic Adrenalectomy Indeterminate or probably Malignant>6cm : Open radical resection with en bloc dissection of involved adjacent organs Metastatic disease Limited disease – open resection of primary tumor with adjuvant treatment of mets Widespread metastatic disease – palliative treatment

17. ADRENAL MEDULLA TUMORS PHEOCHROMOCYCTOMA AND PARAGANGLIOMA (PPGL ) Tumors arising from the neuroectoderm tissue of adrenal medulla are pheochromocytomas ( PCC ) Those arising from extraadrenal parasympathetic ( carotid body ) and sympathetic ganglia( organ of zuckerkandl ) are paragangliomas ( PGL ) 70% sporadic , remaining –familial Rule of 10 – 10% Malignant, 10% familial , 10%bilateral ,10% Extraadrenal,10%Children .

18. Pathology Highly vascularised with greyish pink on cut surface Areas of necrosis and hemorrhage PCC produce calcitonin, ACTH, vasoactive intestinal polypeptide (VIP) and parathyroid hormone-related protein ( PTHrP )

19. Diagnosis Biochemical – Plasma metanephrine levels 24 hr urinary catecholamine levels Plasma dopamine levels – marker of tumor burden in malignant disease Radiological CT /MRI MIBG scan FDG PET

20. Treatment Medical Management Pre-op control of blood pressure with phenoxybenzamine ( Alpha adrenergic receptor blocker ) 20mg initially followed by 10mg until daily dose of 100 – 160 mg is reached Beta adrenergic blockers if tachycardia or arrhythmias present Post operatively – hypoglycemia and hypovolemic should be checked Surgical Treatment Adrenalectomy

21. Neuroblastoma Most common and deadliest extracranial malignancy in children Derived from primitive nerve cells of sympathetic nervous system of neural crest cells Clinical enigma Peak incidence – 5yrs of age Clinical features – lump across midline of abdomen , Diarrhea , dehydratio due to VIP Metastatic lesions – blue berry muffin lesions , racoon eyes

22. Investigations – MRI > CECT Treatment – Surgical Resection of primary tumor Chemotherapy – Etoposide , Cisplatin

23. GANGLIONEUROMA Ganglioneuromas (GNs) are benign diferentiated tumours of neural crest-derived cells in the autonomic nerves. Sporadic , assosciated with MEN 2b Pathology – admixture of ganglion and schwann cells Sites – Mediastinum , retroperitoneal, Adrenal gland Clinical features – benign , present as non secreting slow growing tumors Usually asymptomatic, <30years , when large in size cause pressure symptoms

24. Diagnosis – CT /MRI – well defined with capsule and calcification Treatment – Surgical excision – laparoscopic method preferred Excellent prognosis

25. THANK YOU