Adrenal mass

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1. ADRENAL MASS DR.SAAD ALYOUSEF QATAR RED CRESCENT RADIOLOGIST

2. Cases FILE NO 276309 COMING TO OUR CENTRE IN ( 5/MARCH/2017 ) FILE NO 21735 COMING IN ( 27/3/2017 )

3. USG REQUEST DIAGNOSIS WELL DEFINED HYPERECHOIC SUPRA RT RENAL MASS ABOUT 6,5 X 4,5 CM

4. CHALLENGING DIAGNOSIS ● Is it hepatic mass? ● Is it renal/ suprarenal mass? ● Is it malignant ? ● Is it functioning?

5. Adrenal masses A. Neoplasm B. Other mass lesion 1. Cortical 1. Granuloma a. adenoma a. tuberculosis b. carcinoma b. histoplasmosis 2. Medullary c. blastomycosis a. pheochromocytoma 2. Bilateral hyperplasia b. neuroblastoma 3. Cyst c. ganglioneuroma a. endothelial (45%) 3. Stromal b pseudocyst (39%) a. lipoma c. epithelial (9%) b. myelolipoma d. parasitic ( hydatid ) 4. Metastasis 4. Hematoma

6. SIZE OF MASS Size — The maximum diameter of the adrenal mass is predictive of malignancy Adrenocortical carcinomas were significantly associated with mass size, with 90 percent being more than 4 cm in diameter when discovered.

7. INDICATORS SUGGESTING POSSIBILITY OF MALIGNANCY Masses > 4cm size tends to be metastasis or primary adrenocortical carcinoma. Irregular shape. Heterogenous appearance. Growth of adrenal mass over time.

8. GROUP I : ADRENAL DISEASE WITH NORMAL FUNCTION Adrenal incidentaloma (AI) is a term applied to an accidentally discovered adrenal mass on imaging performed for the investigation of an unrelated complaint. Adrenal incidentalomas (AIs) are a cluster of different pathologies, the majority of which are benign and non-functioning adrenal adenomas. However, mild hormonal alterations as well as metabolic abnormalities may be present in patients with AIs. Most of these are incidentally detected as adrenal masses. Include : nonfunctional adrenal adenoma or carcinoma, metastasis , lymphoma , myelolipoma , adrenal cyst .

9. Benign non-functioning adenomas The vast majority comprise non-functioning, benign cortical adenomas or hypersecretory tumors (subclinical Cushing’s syndrome, pheochromocytoma , aldosterone-secreting adenoma), primary adrenocortical carcinoma, myelolipomas , cysts and various rare benign tumors . When they occur bilaterally , the most possible diagnoses are metastatic disease, congenital adrenal hyperplasia, bilateral cortical adenomas or infiltrative disease 85 percent of the masses : non fuctioning . 9 percent secreted sufficient cortisol to produce subclinical Cushing's syndrome . 4 percent : pheochromocytomas (less than half caused hypertension) . 2 percent : aldosteronomas .

10. GROUP II : HYPERFUNCTIONING ADRENAL NEOPLASM Adrenal medullary neoplasm Pheochromocytoma Adrenal cortical neoplasm Cushing syndrome. Hyperaldosteronism or Conn sydrome . Hyperandrogenism .

11. Cortisol hypersecretory masses These lesions are characterized by autonomous glucocorticoid production without specific signs and symptoms of Cushing’s syndrome (CS), a condition termed subclinical hypercortisolism (SCS )& may be associated with a high prevalence of arterial hypertension, obesity, impaired glucose tolerance and dyslipidemia, clinical features also shared by the metabolic syndrome . This association is also supported by the fact that these hormonal and clinical features were improved in all patients after surgical treatment .

12. Pheochromocytoma (PHEO) PHEOs are rare, catecholamine-producing tumors arising from the chromafin cells of the adrenal medulla. Their prevalence in the general population is estimated at about 1 or 2 per 100,000 adults. The vast majority of PHEOs are sporadic (about 86%), while the remainder (14%) are associated with familial syndromes, such as neurofibromatosis type 1, von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 1 (MEN1) and 2 (MEN2) Neoplasm of adrenal medulla. Usually unilateral and benign . C/F—paroxysmal headache, palpitation, tachycardia, perspiration, HTN as tumor secretes catecholamines . Clinically suspected in younger patient with hypertension. Rule of 10 : bilateral in 10% malignant in 10% extraadrenal in 10% multicentric in 10% familial in 10%

13. The main clinical feature is hypertension, which is paroxysmal in 48% and persistent in 29%, while 13% of the patients are normotensive. Besides hypertension, a common triad of symptoms comprises attacks of headaches (80%), palpitations (64%) and diaphoresis. Other manifestations frequently misdiagnosed are those related to endocrine gland pathology (CS, hypercalcemia , diabetes mellitus, thyroid carcinoma) or cardiovascular episodes (such as shock, myocarditis, dilated cardiomyopathy, cardiac arrhythmias)

14. HYPERALDOSTERONISM Primary aldosteronism (PA) in its classical form manifests with high aldosterone (ALD), low plasma renin activity (PRA) and hypokalemia, although several reports indicate that normokalemic PA constitutes the most common presentation of the disease . Hypertension, mild or severe, is the common finding in these patients and if hypokalemia is present, nocturia , polyuria, muscle cramps, palpitations or paralysis may occur. Three main etiologies : 1) adrenal adenoma 2) adrenal hyperplasia 3) adrenocortical carcinoma Adrenal adenomas are usually less than 2cm , solitary and eccentric within gland. Adrenal hyperplasia : adrenal glands mildy enlarged and have irregular surface .

15. Primary adrenocortical carcinoma Primary adrenocortical carcinoma is rare. can be functional or non-functional . The clinical manifestation involves symptoms related to adrenal hypersecretion , such as hypercortisolism (more common), as well as high sex hormones or ALD hypersecretion (presented with CS, hirsuitism , acne, oligo - or amenorrhea, increased libido in the case of androgens, gynecomastia in the case of estrogens , hypokalemia-related symptoms, in the case of elevated aldosterone)

16. Metastatic cancer Metastatic disease to the adrenal glands can occur in a wide array of malignancies . The most frequently reported primary cancers that metastasize to the adrenals are lung (usually the non-small cell type) and liver (hepatocellular carcinoma)

17. Adrenal myelolipoma rare, benign usually hormonally inactive neoplasm of the adrenal cortex and consisting of mature adipose cells and haematopoietic elements in varying proportions.It is asymptomatic in most cases, although, especially the large ones can present with abdominal pain or retroperitoneal bleeding. Adrenocortical dysfunction occurs in 10% and may present as Addison’s disease, Cushing’s disease, hyperandrogenism or hypertension due to catecholamine or aldosterone secretion

18. GROUP III : ADRENAL HYPOFUNCTION No specific syndrome has been described. May be due to adrenal destruction or inadequate pituitary stimulation. CAUSES : autoimmune disorders infections – fungal and TB Adrenal hemorrhage Sarcoidosis Drugs: inhibit cortisol synthesis( ketoconazole , etomidate ) , or increase cortisol clearane (barbiturates and phenytoin.)

19. Radiographic features Imaging plays a key role in assessing the vast number of incidental adrenal lesions, the majority of which are adrenal adenomas. Correlation with previous imaging is often useful, as a lesion which has not changed over some years is unlikely to be malignant. General They can be divided into those that have typical or atypical appearances. Typical adenomas are: small: <3 cm homogeneous and low density Atypical features include: haemorrhage calcification necrosis no fat large if >4 cm: 70% malignant (excluding adrenal myelolipomas which are easily recognised due to fat, and pheochromocytomas which are usually recognised biochemically) if >6 cm: 85% malignant

20. CT Routine CT protocol for adrenal imaging NCCT abdomen CECT abdomen (70 secs delay) Delayed scan (after 15 minutes) Computed tomography (CT) is the imaging modality of choice for evaluating adrenal glands morphology and masses associated with it. High resolution CT of upper abdomen, using 1-3mm thick slices to reduce the volume averaging, is most accurate technique for indentifying adrenal lesions. Contrast-enhanced CT and delayed images help in further characterization of the lesions. 100-150ml of contrast is injected at a rate of 3mlper second and images are aquired at 70sec and 15 min after contrast injection.

21. BENIGN Size Small No change Smooth margin Homogenous NCCT : HU < 10 CECT----- Mild & rapid enhancement Quick wash-out MALIGNANT Size >4cm Change in size Irregular shape Heterogenous NCCT : HU > 10 CECT----- Heterogenous & vigorous enhancement Prolonged wash-out

22. NON CONTRAST CT

23. CONTRAST ENHANCED CT

24. The enhancement washout = (43 - 22) : (43 - 9) = 62% indicating a fast washout characteristic of an adenoma.

25. MRI MRI of the adrenals is the modality of choice for further characterization of adrenal lesions. MR parameters should include T1-and T2-weighted sequences along with chemical shift imaging. T1 weighted signal show normal adrenal as low signal against high signal fat. Most tumor show high signal on T2W and low signal on T1W image. Contrast enhanced dynamic MRI used in d/d of adenoma, metastasis, granulomas and pheochromocytoma Chemical shift MR used in d/d of adenoma and metastasis: adenoma – high lipid content

26. MRI IN DIFFERENTIATING BENIGN VS MALIGNANT: Various MR parameters used are : T1 T2 Enhancement pattern. Chemical shift characteristics

27. Use of chemical shift imaging to differentiate adenoma and metastasis CT scan

28. Conclusion Most adrenal masses are incidentalomas and amongst them, adenomas are most common, which can be functioning or non-functioning. Some adrenal masses may have pathognomonic CT features such as myelolipoma , cysts, lipid-rich adenomas and malignant masses but most incidentalomas have nonspecific morphologic features. Most adrenal adenomas are lipid-rich and can be correctly diagnosed on chemical-shift MR imaging or unenhanced CT. Most lipid-poor adenomas can be accurately characterized on delayed enhanced CT.

29. References UPTODATE.COM RADIOPEDIA.COM https :// radiopaedia.org/articles/adrenal-adenoma http://www.hormones.gr/520/article/adrenal-incidentaloma:- a-diagnostic-challenge%E2%80%A6.html http:// www.cancer.net/cancer-types/adrenal-gland-tumor/diagnosis

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