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adrenal tumor.pptx

  1. Adrenal Gland Disorders By LoritaFurtado
  2. Overview of Adrenal Glands • The adrenal glands (suprarenal glands) are paired endocrine glands situated over the medial aspect of the upper poles of each kidney. They secrete steroid and catecholamine hormones directly into the blood. • The adrenal cortex produces 3 main types of steroid hormones namely 1. Mineralocorticoids- (such as aldosterone)-help in the regulation of blood pressure and electrolyte balance. 2. The glucocorticoids cortisol and cortisone-help in regulation of metabolism and immune system suppression. 3. The androgens that are converted to fully functional sex hormones in the gonads and other target organs] The Chromaffin cells of the medulla produce Catecholamine namely adrenaline and noradrenaline which produce a Rapid response throughout the body in stress situations. The adrenal medulla is driven by the sympathetic nervous system.
  3. Adrenal Hormones related disorders CORTICOSTEROID OVERPRODUCTION Cushing's syndrome- is the manifestation of glucocorticoid excess. • It can be the result of a prolonged treatment with glucocorticoids or be caused by an underlying disease which produces alterations in the production of cortisol. Primary aldosteronism Results when the zona glomerulosa produces excess aldosterone. • Causes for this condition are bilateral hyperplasia (excessive tissue growth) of the glands, or aldosterone-producing adenomas (a condition called Conn’s Syndrome). Primary aldosteronism produces hypertension and electrolyte imbalance, increasing potassium depletion and sodium retention.
  4. Adrenal Hormonesrelated disorders  .ADRENAL INSUFFICIENCY • Diseases are classified as primary adrenal insufficiency (including Addison’s Disease and genetic causes) directly affect the adrenal cortex. If a problem that affects the HPA axis arises outside the gland, it is a secondary adrenal insufficiency.  Addison's disease • Addison's disease refers to primary hypoadrenalism, which is a deficiency in glucocorticoid and mineralocorticoid production by the adrenal gland  Congenital adrenal hyperplasia is a congenital disease in which mutations of enzymes that produce steroid hormones result in a glucocorticoid deficiency and malfunction of the negative feedback loop of the HPAaxis. In the HPAaxis, cortisol (a glucocorticoid) inhibits the release of CRH and ACTH, hormones that in turn stimulate corticosteroid synthesis. As cortisol cannot be synthesized, these hormones are released in high quantities and stimulate production of other adrenal steroids instead. The most common form of congenital adrenal hyperplasia is due to 21- Hydroxylase deficiency. 21-hydroxylase is necessary for production of both mineralocorticoids and glucocorticoids, but not androgens. Therefore, ACTH stimulation of the adrenal cortex induces the release of excessive amounts of adrenal androgens, which can lead to the development of ambiguous genitalia and secondary sex characteristics.
  5. CUSHINGSYNDROME It’s a constellation of symptoms associated with cortisol excess (glucocorticoid). Cushing disease is caused by a pituitary gland tumor (usually benign) that over-secretes the hormone ACTH, thus overstimulating the adrenal glands' cortisol production. Cushing syndrome refers to the signs and symptoms associated with excess cortisol in the body, regardless of the cause.
  6. Diagnosisof Cushing’sSyndrome • Obtain a careful history to exclude exogenous glucocorticoid use. • Tests to obtain the diagnosis are: • Urine free cortisol (UFC) (at least two measurements) - 24-hr urine sample • Late-night salivary cortisol (two measurements)- Cortisol levels in blood are normally elevated at 8 A.M. and decrease to less than 50% by midnight except in infants and young children in whom a diurnal rhythm is not always established. In patients with Cushing syndrome this circadian rhythm is lost, and cortisol levels at midnight and 8 A.M. are usually comparable • Serum ACTH level ,FBC • Tests to diagnose –Hyperglycemia, Hyperlipidemia ,• Hypokalemia, Metabolic alkalosis • U/E – low K • DST( Dexamethasone suppression test) Dexamethasone is an exogenous steroid that provides negative feedback to the pituitary to suppress the secretion of ACTH. Dexamethasone binds to glucocorticoid receptors in the pituitary gland resulting in regulatory modulation. The test is given at low (usually 1–2 mg) and high (8 mg) doses of dexamethasone, and the levels of cortisol are measured to obtain the results.
  7. Managementof CushingDisorder MEDICAL • Steroidogenic inhibition Eg: Mitotane, Ketoconazole • Neuromodulatory treatment Eg:Bromocriptine, Valproic acid • Glucocorticoid receptor Eg:antagonist RU486 SURGICAL • Trans sphenoidal microadenomectomy • Pituitary radiation • Bilateral total adrenolectomy • Adrenal adenoma and carcinoma • Surgical removal Ectopic ACTH Syndrome • Surgical removal of the ectopic tumor • Radiotherapy
  8. Addison’s Disease • Addison's disease is serious chronic disease, caused by partial or absolute abnormality of hormonal function of the adrenal cortex . • Arises when cortisol levels are not sufficient to meet the needs of the body. • Causes • Autoimmune: - Isolated autoimmune adrenalitis (30- 40%) • Polyglandular syndrome 1 &2 (60- 70%) • Infection:TB, HIV, CMV, cryptococcosis, histoplasmosis, coccidioidomycosis • AIDS • Metastases • Bilateral adrenalectomy
  9. Diagnosis & Management DIAGNOSIS • In the blood analysis: lymphocytosis, eosinophilia, erythrocyte sedimentation rate is decreased, Hypoglycemia • The electrolyte: hyponatremia, hypochloremia, hyperkaliemia • Baseline Cortisol and ACTH levels should be obtained in the early morning The content of ACTH is increased; The content of cortisol is decreased • Narrow cardiac silhouette on CXR, Low voltage EKG MEDICAL MANAGEMENT • Glucocorticoid therapy- Hydrocortisone therapy • Mineralocorticoid replacement-fludrocortisone • Adrenal androgen replacement -in patients with lack of energy, and in women with loss of libido.
  10. Addisonian Crisis Clinical Manifestations • Severely low blood pressure (shock) • Hyperkalemia • Hyponatremia • Hypoglycemia • Hypercalcemia • Unexplained fever, diarrhea, vomiting • Coma and death • Precipitated by infection, surgery or intercurrent disease Management of Addisonian Crisis • It is a medical emergency • IV fluid (normal saline 1 L/h with continuous cardiac monitoring and 10% dextrose) • Hydrocortisone 100 mg bolus followed by 100–200 mg hydrocortisone over 24 h infusion or i.v doses until GI symptoms improve then start oral therapy • Mineralocorticoid replacement can be initiated once the daily hydrocortisone dose has been reduced to <50 mg • Treat precipitating cause
  11. Adrenal tumor • An adrenal tumor or adrenal massis any benign or malignant neoplasms of the adrenal gland, with a usual tendency to overproduce endocrine hormones. • When adrenal gland tumor produce too much of hormone it is called a “functioning tumor” and when it does not produce hormones is called a “nonfunctioning tumor.” Types of Adrenal Tumors Incidentalomas •Is an adrenal tumor found by coincidence without clinical symptoms or suspicion.
  12. Types of adrenal gland tumors- Tumors of the adrenal cortex • Adrenocortical adenoma/Benign adenomas • are encapsulated, well-circumscribed, solitary tumors with solid, homogeneous yellow-cut surface . • are relatively small, usually less than 2 inches in diameter and most people with this type of tumor have no symptoms. These tumors usually occur on only one adrenal gland, but they can appear on both glands in rare instances. • Functional adrenocortical adenomas are surgically curable. • Adrenocortical carcinomas • are usually much larger than benign adenomas. • They frequently invade large vessels, such as the renal vein and inferior vena cava, as well as metastasizing via the lymphatic and through the blood to the lungs and other organs. The most effective treatment is surgery, although this is not feasible for many patients, and the overall prognosis of the disease is poor. Chemotherapy, radiation therapy and hormonal therapy may also be employed in the treatment of this disease.
  13. Typesofadrenalglandtumors-Tumorsoftheadrenalmedull Neuroblastoma • is an aggressive cancer of immature neuroblastic cells ; one of the most common pediatric cancers and typically presents with a rapidly enlarging abdominal mass. • Although the tumor has often spread at the time of diagnosis, this cancer is unusual in that many cases are highly curable when the spread is limited to the liver,skin and/or bone marrow. • Related, but less aggressive tumors are ganglioneuroblastoma and ganglioneuroma. • Treatment of neuroblastoma includes surgery and radiation for localized disease, and chemotherapy for metastatic disease. Pheochromocytoma • is a neoplasm composed of cells similar to the chromaffin cells • Occur in patients of all ages, and may be sporadic, or associated with a hereditary cancer syndrome. The most clinically important feature of Pheochromocytoma is their tendency to produce large amounts of the catecholamine hormones leading to potentially life-threatening high blood pressure or cardiac arrhythmias and numerous other symptoms. • Only 10% are malignant. Signs and symptoms are The five P’s:-Pressure (HTN) , Pain (Headache), Perspiration, Palpitation, Pallor, Paroxysms (6th P!)
  14. Adrenal Tumors CAUSES • Medullary thyroid carcinoma • Parathyroid Hyperplasia • Emotional and physical stress. • General factor • Increased or Decreased secretion of Hormone. SIGN AND SYMPTOMS • Hypertension ((320/200mm.Hg) • Hypermetabolism • Hyperglycaemia • Head ache, Visual Disturbances, Nervousness • Abdominal pain • Polyuria • Psychotic Behavior, Depression and Emotional Upset. • Allergic Reactions
  15. Adrenal Tumors Staging of Adrenal Carcinoma The WHO classification of 2004 is based on the McFarlane Classification & defines four stages: • I stage –Tumor < 5 cm • II stage – Tumor >5 cm • III stage – Locally invasive tumors • IV Stage – Tumor with distant metastasis • DIAGNOSTIC EVALUATION • History collection andPhysical examination • Biochemical evaluation- Morning & midnight plasma cortisol measurement • Dexamethasone suppression test(DST) is used to assess adrenal gland function by measuring how cortisol levels change in response to an injection of dexamethasone. • 24 hr urinary cortisol measurement • Serum potassium, plasma aldosterone & plasma renin activity • Abdominal imaging, CT scan,MRI scan • Adrenal vein catheterization
  16. MANAGEMENT of Adrenal Tumors MEDICAL • Alpha- Adrenergic Blocking Agents: Inhibit the effects of Catecholamines on blood pressure. • Catecholamine Synthesis Inhibitor: Used pre operatively or for long term management of in operable tumors. • Beta Adrenergic blocking agent: Propranolol - Used for patient with cardiac dysarhythmias or those not responsive to alpha adrenergic blocking agent. • Corticosteroid replacement: To prevent adrenal insufficiency SURGICAL MANAGEMENT • Unilateral or Bilateral Adrenalectomy Chemotherapy and Radiation Therapy in case of neoplasms
  17. Hereditary disorders associated with adrenal tumors • Von Hippel–Lindau disease, a mutation of the VHL1 tumor- suppression gene associated with many types of tumor, including pheochromocytoma. • Multiple Endocrine Neoplasia, a family of syndromes in which genetic abnormalities contribute to the development of endocrine tumors
  18. References • • • gland-diseasescushing-syndromeaddison-disease-and- pheochromocytoma?qid=bcee84b3-1e25-4310-950e- b8651a13ac5a&v=&b=&from_search=2 • 231158894?qid=da9659be-252b-438f-91b5- 4f06b4c03d82&v=&b=&from_search=1 Thank You