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Approach to Adrenal Incidentalomas

Approach to Adrenal Incidentalomas. Moayad Assiri Urology R4 KAUH. Definition. Prevalence. Causes led to it discovery. DDx. Diagnostic approach -Hormonally. -Imaging. -? FNA. Management approach.

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Approach to Adrenal Incidentalomas

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  1. Approach to Adrenal Incidentalomas Moayad Assiri Urology R4 KAUH

  2. Definition. • Prevalence. • Causes led to it discovery. • DDx. • Diagnostic approach -Hormonally. -Imaging. -? FNA. • Management approach. • F\U. • Summary. • References.

  3. (Clinically inapparent adrenal masses). Definition • adrenal tumor not suspected prior to radiological studies led to it discovery. • A disease of modern technology. J Endocrinal Metab Clin North Am 1997 • Except: -Pt w known non adrenal malignant tumor. -pt known HTN.

  4. Incidence of adrenal masses is 9 - 12% in autopsies. • Incidence of AI is 2- 5 %. • > • Rt > lt . • Bil 2 – 10 %. NATIONAL INSTITUTES OF HEALTHManagement of the Clinically Inapparent Adrenal Mass (Incidentaloma). Final Statement. July 16, 2002

  5. Adrenal Incidentalomas Study - Chi Mei Medical Center • 1998 Jan – 2002 Dec • Abdominal CT scan 70,101 in 5 years • 127 cases of AI in 256 cases of adrenal tumors • Incidence of AI: 0.18% • Hormonally active: 4%

  6. Mayo clinic 1985-1990 • 61,054 CT scans performed . • Adrenal mass in 2066 pts ( 3.4%). • 16.5% were present incidental ( <1 cm ). • Overall, AI 259 pts ( 0.4% ) • 50% metastatic ca. • 25% other known lesions. • 7.5% symptomatic tumors.

  7. Causes led to discover IA • 342 cases, 136 men and 206 women • Reasons for CT scan: nonspecific abdominal pain, 60% known abdominal or thoracic disease, renal problems, musculoskeletal pain Acta Endocrine (copenh)1989

  8. Age-dependent occurrence of adrenal adenomas • 1% in age < 30 y. • 3 -7 % in age > 50 y. • Compensatory growth in response to local ischemic damage of arteriosclerotic disease • Higher number of diagnostic procedures performed in aged patients N Engl J Med, 1990 Increases with Age

  9. Age-dependent occurrence of adrenal cortical adenomasautopsy studies with total of 57,262 subjects Endocrine and Metabolism Clinics of North America 2000; 29(1):159-185

  10. Clinical characteristics • Per definition ,NO clinical symptoms or signs of adrenal disease should be present at the time of Dx. • More detailed Q and careful PE might reveal evidence of subtle hormonal excess; -recent w.t gain -skin atrophy. -episodic of headaches.

  11. Àdrenal incidentaloma • Majority are neither function nor malignant 70-90%. • The malignant tumors may and may not secrete hormonal. • The 3 mains hormone-producing tumors: • Cortisone producing adenoma SCCS 11%. • Pheochromocytoma 7%. • Aldoestrone producing adenoma 2%.

  12. PREVALENCE OF ADRENAL INCIDENTALOMAS IN DIFFERENT SERIES _______________________________ Type of Tumor Mantero et al Al-SIE Othorst _______________________________________________ Nonhypersecretory tumors 76.0 74.0 70-94 Hypersecretory tumors Cortisol-secreting adenomas 14.0 9.2 0-14 Aldosteronomas 1.0 1.4 0-7 Pheochromocytomas 3.4 4.2 0-11 Adrenal carcinomas 1.0 4.0 0-25 Myelolipomas 2.0 3.0 7-15 Cysts 1.0 1.9 4-22 Ganglioneuromas 1.0 1.5 0-6 Metastases Unselected patients 1.0 0.7 0-21 Oncological patients 32-73 ________________________________________ AI-SIE: Adrenal Incidentaloma Study Group of the Italian Society of Endocrinology Prevalence (%) Endocrine and Metabolism Clinics of North America 2000; 29(1):107-126

  13. Adrenal cancer • Benign Adenoma 50% • Cyst 10% • Myelolipoma 10% • Pheochromocytoma 10% • Metastases 6-30% • Adrenal Cancer 0.01% The incidental adrenal mass. Am J Med 1996

  14. Benign nonfunctioning Adenoma Adrenolipoma Amyloidosis Cyst / Pseudocyst Ganglioneuroma Granuloma Infection Hamartoma HemangiomaLeiomyoma Lipoma Myelolipoma Malignant nonfunctioning Angiosarcoma Ganglioneuroblastoma Leiomyosarcoma Malignant schwannoma Metastatic carcinoma Malignant melanoma Malignant lymphoma Adrenocortical carcinoma Differential Diagnosis of Adreanl Incidentalomas Endocrine and Metabolism Clinics of North America 2000; 29(1):159-185

  15. Hyperfunctioning mass - Pheochromocytoma - Preclinical Cushing’s syndrome - Primary aldosteronism - Nodular hyperplasia - Congenital adrenal hyperplasia - Masculinizing or feminizing tumor - Primary malignancy (Adrenocortical carcinoma) Pseudoadrenal mass - Mistaken vasculature - Liver - Lymph nodes - Pancreatic mass - Spleen - Renal mass - Stomach mass - Technical artifact Differential Diagnosis of Adreanl Incidentalomas Endocrine and Metabolism Clinics of North America 2000; 29(1):159-185

  16. After incidental discovery of an adrenal mass, 2 questions should be addressed • Functional status hormonally active even in the absence of a classic clinical presentation • Malignant potential any chance that the mass is malignant

  17. Important consideration • Trying not to miss important pathology. • Trying no to subject the pt to unnecessary procedure or surgery. • Cost effect.

  18. 1-Endocrinology investigationsignificant endocrine activity increase with increase tumor size

  19. Subclinical Cushing’s Syndrome • Most frequent hormonal abnormality (5 – 20%) • Autonomous cortisol secretion without stigmata of Cushing’s syndrome • Diagnostic criteria not yet clearly defined • 10-20 mg/day • Controlled Primarily By ACTH • Negative and Positive Feedback Between Cortisol and ACTH at Pituitary and Hypothalamic Levels

  20. Dx • IncreasedUrinary Free Cortisol (UFC) • Unsuppressed Serum Cortisol Levels After 1-mg OvernightDexamethasone • Low ACTH Levels • No Clinical Signs Of Cushing Syndrome Mantero F, Masini AM, Opocher G, Giovagnetti M, Arnaldi G. Horm Res 47:284–289, 1997

  21. Dexamethasone Suppression Test • Low-dose: 1mg dexamethasone at 2300, followed by assay of cortisol at 0800 the following day • Cortisol > 3 mcg/dl suggests autonomous secretion • High-dose: 8 mg dexamethasone at 2300 with assay of cortisol at 0800 the next day • Cortisol > 1 mcg/dl indicates autonomous secretion • Reduces false positives • “Intermediate-dose”: 2 – 3 mg dexamethasone at 2300, followed by cortisol at 0800 • Cortisol > 3 mcg/dl reportedly suggestive of autonomy • Easier to perform overnight suppression rather than 2-day oral load

  22. Association with Metabolic Syndrome? • May contribute to, and worsen, hypertension, obesity , diabetes, dyslipidemia and osteoporosis • 1990’s Italian multiinstitutional study collection 1004 patients with adrenal incidentaloma • 41% prevalence HTN • 10% prevalence DM • 28% prevalence obesity

  23. Pheochromocytoma • Approximately 5-7 % of adrenal incidentalomas. • 0.5% among screened hypertensive pt’s • Can be clinically silent and not suggested by history • Mixed recommendations on diagnostic testing

  24. Dx • 24-h Urinary fractionated catecholamine excretion or free metanephrines. • 24 hour urine fractionated metanephrines recommended by some as first diagnostic test • Especially in incidentalomas without radiographic features suggestive of pheochromocytoma • Sensitivity and specificity between 91 – 98% • Increased even further when fractionated catecholamines added

  25. Plasma Free Metanephrines • Plasma free metanephrines • Used when imaging characteristics and other pre-test probability of pheochromocytoma are high, but 24 hr urine normal • Due to high sensitivity (97 – 100%) but low specificity (85 – 89%, ) of test • Negative result quite effective at ruling out

  26. Which Is Better? Catecholamine-Secreting Tumors Histologically Proven In 31 of 340 Patients: • Sensitivity Of Plasma Free Metanephrine 97% Specificity 98% • Sensitivity Of Urinary Total Metanephrine And Catecholamines 90%; Specificity 85% Sawka et al. JCEM 88(2): 553-558. 2003.

  27. Aldosteronism (Conns Sx) • Quite rare cause <1% • Most have already been diagnosed before become large enough to be detected by CT/MRI • Should be evaluated in all hypertensive patients with incidentaloma • Serum potassium not sufficient, but should be included • Common to have normokalemia with aldosterone producting adrenal tumor 7 – 38%.

  28. Hypertension Headache Weakness And/Or Fatigue Paresthesias Muscle Cramps Polyuria, Polydipsia, Nocturia Arrhythmias Signs and Symptomsin Primary Hyperaldosteronism

  29. Aldosterone Production • 100-150 ug/day • Stimulated by the Renin-Angiotensin System  Decreased Perfusion Pressure • Hyperkalemia Stimulates and Hypokalemia Inhibits Aldosterone Secretion • ACTH Acutely May Stimulate Aldosterone Secretion

  30. Dx • Plasma aldosterone/plasma renin activity ratio greater than 30 highly suggestive • Especially with plasma aldosterone >15 ng/dl • Confirm with aldosterone suppression test • IV 0.9 NS infusion – 2L over 2 hrs, with supine aldosterone >10 suggestive • Can also do 24 hour urinary aldosterone while patient orally ingests 5000 mg sodium/day x 3 days • Urinary aldosterone >14 mcg/24º

  31. Appropriate screening tests should be performed if the patient has clinical features that are suggestive of increased adrenal function

  32. Stepwise diagnostic approach • Step I : • 1- 24-h Urinary catecholamine excretion or free metanephrines. • 2- Serum cortisol after dexamethasone suppression 1mg at 11 pm PO. • 3-HTN , Serum K and – PAC\PRA ratio.

  33. Stepwise diagnostic approach • Step II : confirmatory test. • 1- MRI . CT scan or 123-I-MIBG scintigraphy or 131 I-MIBG. • 2- high-dose dexamethasone 8mg suppression test. If +ve .CRH test analysis of diurnal cortisol secretion , and 24-h urine free cortisol. • 3- confirm with aldosterone suppression test. And 24-h urine aldosterone .

  34. Stepwise diagnostic approach Step III. Revaluation -repeat screening tests after 2 years in patient with a tumor size of > 3 cm.

  35. 2- Imaging

  36. Imaging Characteristics • CT Scan • Hounsfield Scale • Measures xray attenuation • Each number represents a shade of grey, with air and bone • Water as zero • Air = -1000 • Bone = +1000

  37. CT scan • Attenuation On CT Scan Is Measured In Hounsfield Units (HU) • Lipid-Rich Masses Are Usually Benign • Lipid-Rich Lesions Have Low Attenuation • Low Attenuation Lesions Have Low HU Values

  38. Computed Tomographic Scans • Unenhanced CT: Adenomas: < 10 HU Malignancies: > 18 HU Sensitivity: 73% Specificity: 96% • Delayed Enhanced CT: Adenomas: < 30 HU Malignancies: > 30 HU Sensitivity 95% And Specificity: 100%

  39. Adrenocortical carcinoma Size large, typically > 4 cm Shape irregular with unclear margins Texture inhomogenous with mixed densities compared with liver Laterality usually solitary and unilateral CT > 10 HU without contrast and >30-40 HU 30 min after contrast administration MRI hyperintense to liver on T2-weighted image Presence of necrosis, hemorrhage, or calcifications-- common Growth usually rapid

  40. Adrenal cancer CT Contrast-enhanced CT scan through the abdomen of a 56-year-old man reveals a complex solid and cystic, calcified mass (arrow) in the right suprarenal fossa extending into the adjacent liver. The tumor proved at surgery to be a carcinoma of the adrenal cortex. .

  41. Malignant Potential • Imaging phenotype and mass size are two major predictors of malignancy • Additional test that may be preformed to predict malignancy include image-guided needle biopsy and iodocholesterol scintigraphy • Hormonal evaluation is not predictive of malignancy • Approximately 41% of 1,891 published cases of adrenocortical carcinoma, was not hormone-secreting

  42. size • The probability that an AI is ACC is increases with increasing size of the lesion. • 90% of all ACC is > 6 cm in size. • The prevalence of ACC related to size of tumor • 2% in tumors < 4 cm. • 6% in tumors 4-6 cm. • 25% in tumor > 6 cm. • Resection all tumor > 4 cm - 8 benign tumors removed for each cancer resected.

  43. Imaging Phenotypes of Adrenal Incidentalomas Adrenal cortical adenomas Size small, typically < 3 cm Shape round to oval with smooth margins Texture homogenous and low density compared with liver Laterality usually solitary and unilateral CT < 10 HU without contrast and <37 HU 30 min after contrast administration MRI isointense to liver on T2-weighted image Presence of necrosis, hemorrhage, or calcifications-- rare Growth usually stable size over time or very slow growth

  44. Adrenal adenoma hypodense Abdominal CT showing a 1.5-cm round hypodense left adrenal cortical adenoma (arrow).

  45. Pheochromocytoma Size large, typically > 3 cm Shape round to oval with smooth margins Texture inhomogenous with cystic texture Laterality usually solitary and unilateral CT >10 HU without contrast and >40 HU 30 min after contrast administration, usually vascular, marked enhancement MRI marked hyperintense compared with liver on T2-weighted image Presence of necrosis, hemorrhage, or calcifications-- hemorrhage and cystic necrotic areas common Growth very slow

  46. Pheochromocytoma silent Abdominal CT showing an 8-cm left adrenal mass (arrow) discovered incidentally. Note the vascular and inhomogenous imaging characteristics of this mass. Biochemical evaluation was consistent with a clinically silent pheochromocytoma, which was confirmed at surgery..

  47. MRI of the abdomen shows a 4.5-cm right adrenal pheochromocytoma (arrows). Upper panel: T1-weighted image. Lower panel: T2-weighted image shows increased signal intensity typical of a pheochromocytoma.

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