0. Adrenal—Incidentals, Pearls, Pitfalls
Clive Grant, MD
Mayo Clinic
CP Grant, CS MN/KK

1. Disclosures
None

2. Adrenal Case HPI 55 y/o female
1 yr ago: L mast, – SLN for 3.5 cm infiltrating ductal carcinoma; ER, PR +
4 cycles AC chemotherapy; Tam
Energy and WBC (2,600) did not return to nl
Home MD further investigated

3. Adrenal: Patient Eval Meds: Tamoxifen PSH: 2 back operations; appy
PMH: Well except breast Ca
Exam: VS nl Wt 120 lbs
Chest: mast site neg
Nodes: all neg
Abd, pelvis: neg
Labs: Lytes, glu, Cr, LFTs, CXR nl

4. CT Imaging
CT: 6 cm mass replacing R adrenal. L adrenal nl; no hepatic masses; no abdominal lymphadenopathy

5. Adrenal Mass: Evaluation
What further testing?
Serum, urine aldosterone
AM, PM cortisol
24-hr urine “mets and cats”
1 mg overnight dex suppression
CT-directed biopsy
Bone scan
PET scan

6. Adrenal Mass: Testing Test Results Aldo Cortisol Pheo Dex supp Bx
Bone scan
PET scan
Results
Not Done
Normal
Small sample: positive for malignancy, c/w breast ca
No mets

7. PET Scan
No abnormal uptake except a large mass in the right adrenal gland, consistent with malignancy.

8. Adrenal Mass: Management
Options?
Switch to different br ca drug
Laparoscopic adrenalectomy
Open anterior adrenalectomy
Right adrenal radiation

9. Adrenal Cancer: Treatment
Surgical
Open anterior right total adrenalectomy
Pathology
7.5 cm, 115 gm adrenocortical carcinoma; no extra-adrenal invasion

10. Adrenal Incidentaloma Objectives
Following this presentation, you should
Understand the definition and frequency of adrenal incidentaloma
Be prepared to evaluate an incidentaloma and make management recommendations
Understand the pitfalls and nuances of the management algorithm

11. Adrenal Incidentaloma Definition
Criteria
1 cm diameter
Well defined
Exclude
Suspected hormonal hyperfunction
Prior/concurrent malignancy
Localized tumor symptoms/signs
Constitutional symptoms of malignancy
CP Grant, CS MN/KK

12. Adrenal Incidentaloma Historical
Autopsy series
1.4-15% incidence
Average size 1 cm
Frequency increases with age
Problem recognition
1982 Geelhoed, 20 patients
1983 Prinz, 9 patients
CP Grant, CS MN/KK

13. Adrenal Incidentaloma Nl Anatomy, Early CT
Nl adrenals
Incidentaloma, 1983
CP Grant, CS MN/KK

14. Adrenal Incidentaloma Age Dependence
CP Grant, CS MN/KK

15. Adrenal Incidentaloma Algorithm
Adrenal tumor
Assessment
for malignancy
Evaluation for
hyperfunction
Screen
Observe
Excise
CP Grant, CS MN/KK

16. Adrenal Incidentaloma Endocrine Hyperfunction
Possible Occult Functioning Tumors
Pheochromocytoma
Aldosteronoma
Cushing’s adenoma
CP Grant, CS MN/KK

17. Adrenal Incidentaloma Pheochromocytoma
Incidentalomas
5.1% prove to be pheochromocytomas
7% pts with pheos have nl 24-hr urinary fractionated catecholamines
7% have nl 24-hr urinary total metanephrines
99% of functioning pheos have increased levels of one or both of above levels
24 hr urinary mets and cats
CP Grant, CS MN/KK

18. Adrenal Incidentaloma Primary Aldosteronism
Screening
Most frequent cause of 2º hypertension
Morning PAC/PRA
On any antihypertensive except spironolactone
Ratio of  20 and a PAC of 15 ng/dL is positive
CP Grant, CS MN/KK

19. Adrenal Incidentaloma Preclinical Cushing’s Syndrome (PCS)
Definition
Pts who lack typical signs/sxs of hypercortisolism having an incidentaloma with autonomous cortisol secretion
Pts may exhibit side effects of endogenous cortisol secretion
CP Grant, CS MN/KK

20. Adrenal Incidentaloma Frequency--PCS
Study Group--Italian Soc Endocrinology
786 patients
49 (6.2%) with PCS
Continuum of glucocorticoid autonomy
Most adrenal adenomas have functional autonomy
PCS may develop over extended period ( yrs)  repeat hormonal screening at 1-yr F/U
CP Grant, CS MN/KK

21. Adrenal Incidentaloma Evaluation--PCS
Laboratory Criteria
1-mg dex suppression test
8 AM cortisol level > 5 g/dL
Confirm with 2-day low-dose dex suppression test
ACTH assays--most not sensitive enough
CP Grant, CS MN/KK

22. Adrenal Incidentaloma Advantages vs Disadvantages
Op mort/morb
XS surg for benign lesions
Risk of malignancy
Excision
Observation
CP Grant, CS MN/KK

23. Adrenal Incidentaloma Malignant Potential
Potential Distinguishing Criteria
Mass Size
Imaging phenotype
Image-guided needle biopsy
Metastasis
Infection
Iodocholesterol scintigraphy
CP Grant, CS MN/KK

24. Adrenal Incidentaloma Imaging Phenotype
CT characteristics
Density--Hounsfield scale
0 = water 1,000 = bone -1,000 = air
-20 to -150 = adipose
20 to 50 = kidney
If adrenal is < 10, nearly 100% benign adenoma
IV contrast:
Modest enhancement
Rapid washout
CP Grant, CS MN/KK

25. Adrenal Incidentaloma Example
CP Grant, CS MN/KK

26. Adrenal Incidentaloma Example
Precontrast
Postcontrast
Report: 2.8 cm, precontrast HU, immediate postcontrast HU; Delayed HU--Not diagnostic for adenoma (Pathology: adrenal adenoma)
CP Grant, CS MN/KK

27. Adrenal Incidentaloma Imaging Phenotype
CT Characteristics (cont’d)
Shape
Smooth, round/oval vs Irregular
Texture
Homogeneous vs heterogeneous
Laterality
Uni- vs bilateral
Other
Hemorrhage, necrosis, calcifications
CP Grant, CS MN/KK

28. Adrenal Incidentaloma Characteristics
Adenoma
Size--small, typically  3 cm
Shape--round to oval, smooth margins
Texture--homogeneous, low density
Laterality--solitary, unilateral
Contrast enhancement--limited
MR imaging--isointense to liver on T2-weighted image
Necrosis, hemorrhage, Ca2+--rare
Growth--usually stable, very slow growth

29. Adrenal Incidentaloma Characteristics
MRI: Typical appearance of “in phase” and “out of phase” cuts with signal drop out typical for benign adenoma

30. Adrenal Disorders Benign Nonfunctioning Adenoma

31. Adrenal Incidentaloma Imaging Phenotype
Imaging: >25 HU precontrast; enhancing rim
Surgical Dx: pheochromocytoma

32. Adrenal Incidentaloma Characteristics
Pheochromocytoma
Size--large, typically > 3 cm
Shape--round, oval, clear margins
Texture--inhomogeneous with cystic areas
Laterality--solitary, unilateral
Contrast enhancement--vascular, marked
MR--markedly hyperintense on T2
Necrosis, hemorrhage, Ca2+--hemorrhage and cystic necrosis common
Growth--usually slow

33. Adrenal Incidentaloma Pheochromocytoma
“Incidental” pheo
Typical pheo

34. Adrenal Incidentaloma Characteristics
Adrenocortical carcinoma
Size--large, typically > 4 cm
Shape--irregular, unclear margins
Texture--inhomogeneous, mixed densities
Laterality--solitary, unilateral
Contrast enhancement--vascular, marked
MR--hyperintense on T2
Necrosis, hemorrhage, Ca2+--common
Growth--rapid

35. Adrenal Incidentaloma Examples

36. Adrenal Incidentaloma Characteristics
Metastasis
Size--variable, frequently < 3 cm
Shape--oval to irregular, unclear margins
Texture--inhomogeneous
Laterality--often bilateral
Contrast enhancement--vascular, enhancement tumor rim
MR--hyperintense on T2
Hemorrhage/cystic necrotic areas common
Growth--usually slow

37. Adrenal Incidentaloma Imaging Phenotype
Characteristics
Heterogeneous
Irregular border
Enhancing rim
Surgical Dx:
Metastatic colon carcinoma

38. Adrenal Incidentaloma Unusual Tumors
Malignant fibrous histiocytoma
Tb, Addison’s

39. Adrenal Incidentaloma Unusual Tumors
Cystic large cell lymphoma of adrenal

40. Adrenal Incidentaloma Unnecessary Surgery %
Size of tumor (cm)
(115)
(65)
(22)
(12)
(4)
(3)
(1)
32%
12% 6%
Malignant tumors

41. Adrenal Incidentaloma Algorithm
Based on observations:
 10% incidentalomas hyperfunctional, autonomous
< 5% adrenocortical carcinomas
95% adrenocortical cancers > 4 cm
95% cortical adenomas < 5 cm
Imaging phenotype very helpful
FNA rarely indicated

42. Adrenal Incidentaloma Algorithm
H & P
24-hr urine mets, cats
1-mg overnight DST
If  BP: PAC/PRA
Nonfunctional,
< 4 cm
Functioning mass,
> 4 cm
Imaging phenotype
Surgical
resection
Benign
Suspicious
If > 1cm
 size
Repeat imaging
No change--observe

43. Thank you
CP Grant, CS MN/KK