Adrenal Mass. Cynthia Kwong SUNY Downstate Medical Center Grand Rounds October 13, 2016

Transcription

1 Adrenal Mass Cynthia Kwong SUNY Downstate Medical Center Grand Rounds October 13, 2016

2 Case Presentation 65F found to have a 4cm left adrenal mass in 2012 now presents with 6.7cm left adrenal mass PMHx: HTN, arthritis, obstructive sleep apnea, morbid obesity PSHx: lap cholecystectomy, lap appendectomy, radical TAH-BSO, right total knee replacement Previous functional adrenal mass workup negative History of endometrial adenocarcinoma s/p radial TAH-BSO ECHO: EF 55-60% Underwent robot-assisted laparoscopic left adrenalectomy Pathology: myelolipoma

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4 Adrenal Anatomy Arterial Supply Superior suprarenal artery branches off of the inferior phrenic artery Middle suprarenal artery branches off of the aorta Inferior suprarenal artery branches off of the renal artery Venous Drainage Right suprarenal vein drains into IVC Left suprarenal vein joins inferior phrenic vein and drains into left renal vein Lymph Drainage Drains into lumbar (aortic and caval) nodes

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8 Physiology

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10 Glucocorticoids Corticotropin releasing factor (CRF) > Adrenocorticotropic hormone (ACTH) secretion (anterior pituitary gland) > Glucocorticoid secretion (cortisol) Stress response Enhances effect of catecholamine signaling on arterial smooth muscle cells Increases cardiac contractility and vascular tone Anti-inflammatory and immunosuppressive Reduces circulating lymphocyte and eosinophil counts Increases neutrophil counts Histamine release suppressed Cytokine and immunoglobulin production decreased

11 Glucocorticoids Catabolic state Upregulation of gluconeogenesis Inhibition of glucose uptake by peripheral tissues Stimulation of lipolysis Insulin resistance

12 Mineralocorticoids Zone Glomerulosa Regulated by renin-angiotensinaldosterone axis Angiotensinogen -> angiotensin I - > angiotensin II Stimulates aldosterone release Downregulation Hypokalemia Increased sodium delivery to distal tubule Aldosterone Acts on renal distal convoluted tubule Promotes sodium and chloride retention Potassium and hydrogen ions secreted into urine Expansion of extracellular fluid volume

13 Adrenal Sex Steroids Androstenedione, DHEA, DHEA-S Physiological effects are generally weak in comparison to gonadal sex steroids Development of secondary sexual characteristics

14 Catecholamines

15 Adrenal Masses

16 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

17 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

18 Cortisol-Producing Adenomas

19 Cortisol-Producing Adenomas History findings Fatigue, depression, sleep disturbances Weight gain Menstrual irregularities Hypertension Glucose intolerance Easy bruising Osteoporosis / fracture with minimal trauma Physical exam findings Central obesity Supraclavicular fat accumulation Dorsocervical fat pat Facial plethora Thinned skin Purple and wide (>1cm) striae Acne Ecchymoses Hirsutism Proximal muscle weakness or wasting

20 Cortisol-Producing Adenomas Goal: demonstrate 3 pathophysiologic derangements of Cushing syndrome Loss of normal diurnal pattern with abnormally high late-night cortisol secretion (late-night salivary cortisol test) Failure to discontinue the production of cortisol despite absence of ACTH stimulation (dexamethasone suppression test) Excess production of cortisol (24-hr UFC test)

21 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

22 Aldosteronoma Conn s syndrome Hypertension, hypokalemia History: Muscle cramping and weakness Headaches Intermittent or periodic paralysis Polydipsia, polyuria, nocturia Drug refractory hypertension (need of >3 agents)

23 Aldosteronoma

24 Aldosteronoma Aldosterone renin ratio (ARR) > 20 Plasma aldosterone concentration (PAC) Plasma renin activity (PRA) Salt loading confirmation test Positive if aldosterone remains elevated

25 Aldosteronoma Adrenal Venous Sampling Indication: older patients, morphologically abnormal glands bilaterally, unilateral microadenomas Corrected aldosterone/cortisol ratio of >4:1 = unilateral source of aldosterone excess

26 Aldosteronoma Treatment Medical management: spironolactone, eplerenone Surgical management: laparoscopic adrenalectomy

27 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

28 Pheochromocytoma Found in % of hypertensive patients History Severe hypertension Tachycardia, palpitations Cardiac arrhythmias Anxiety attacks Weight loss Sweating Family history of pheochromocytoma, MEN type 2, VHL

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30 Pheochromocytoma Biochemical Evaluation 24-hour urine collection - catecholamines and total/fractionated metanephrines Plasma fractionated metanephrines and normetanephirines Genetic Testing RET, VHL, subunits of succinate dehydrogenase genes

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32 Pheochromocytoma Pre-operative management Phenoxybenzamine 10mg BID: alpha-adrenergic (long-acting) blockage for 1-3 weeks Other meds: doxazosin, metyrosine (alpha-methyltyrosine) Beta-blockade Indications: persistent tachycardia, extra-systoles, arrhythmias Can only be given in setting of adequate alpha-adrenergic blockade Propranolol 10-40mg q6-8hr Calcium channel blockers can be used for preoperative and intraoperative blood pressure control Encourage liberal fluid and salt intact

33 Pheochromocytoma Intra-operative management Arterial line and central line for monitoring Hypertension : nitroprusside, nicardipine, nitroglycerin, phentolamine Tachyarrhythmia : esmolol Ventricular arrhythmia : lidocaine Hypotension after pheochromocytoma removed -> crystalloid and alpha-adrenergic agonists

34 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

35 Adrenocortical Carcinoma Rare tumor, annual incidence approximately 1 : 1,000,000 Ages 40-50, no gender predilection >50% are functional cortisol, aldosterone, sex steroids CT features: heterogeneity, irregular borders, central necrosis, invasion of adjacent structures Treatment: radical open resection with en bloc resection of adjacent organs and/or regional lymphadenectomy Local recurrence and metastases typically occur within 2 years Chemotherapy : mitotane

36 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma Myelolipoma

37 Metastatic lesion Cancers that metastasize to the adrenal gland Lung cancer Breast cancer Melanoma Renal carcinoma Adrenal matastatectomy is rarely indicated. Patients with bilateral adrenal metastatic lesions should undergo evaluation for adrenal insufficiency

38 Adrenal Masses Functional Cortisol producing adenoma (Cushing s syndrome) Aldosteronoma (Conn s syndrome) Pheochromocytoma Malignant Adrenocortical carcinoma Metastatic lesion Other Adrenal cyst Ganglioneuroma: rare, benign, asymptomatic tumor of neural crest origin Myelolipoma

39 Adrenal Incidentaloma Incidence 8.7% (autopsy series) Answer 3 questions: Is it functional? Does it have radiologic characteristics suggestive of a malignant lesion? Does the patient have a history of malignancy? RULE OUT pheochromocytoma prior to any attempted resection or biopsy

40 Adrenal Incidentaloma

41 Adrenal Incidentaloma If incidentaloma does not fulfill criteria for surgical resection: Radiologic re-evaluation at 3-6 months, then annually for 1-2 years Hormonal evaluation at time of diagnosis, then annually for 5 years

42 A 36 year old man has an abdominal CT scan after a motor vehicle crash. No injuries are found, but his blood pressure is 160/100 mm Hg. The CT scan shows a 3cm adrenal mass. Appropriate initial biochemical evaluation should include all of the following EXCEPT A. plasma metanephrines B. plasma aldosterone level C. low-dose overnight dexamethasone suppression test D. plasma renin level E. serum adrenocorticotropic hormone level

43 A 36 year old man has an abdominal CT scan after a motor vehicle crash. No injuries are found, but his blood pressure is 160/100 mm Hg. The CT scan shows a 3cm adrenal mass. Appropriate initial biochemical evaluation should include all of the following EXCEPT A. plasma metanephrines B. plasma aldosterone level C. low-dose overnight dexamethasone suppression test D. plasma renin level E. serum adrenocorticotropic hormone level

44 References AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1) Sabiston Chapter 41 Fischer s Mastery of Surgery Chapter 44: Adrenalectomy Open and Minimally Invasive Cameron Current Surgical Therapy. Adrenal Incidentaloma Netter s Atlas of Human Anatomy. Plate 310, 320 Townsend and Evers: Atlas of General Surgical Techniques. Chapter 8: Adrenals Anterior, Posterior (Open and Laparoscopic)