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Adrenal—Incidentals, Pearls, PitfallsAdrenal—Incidentals, Pearls, Pitfalls
Clive Grant, MDClive Grant, MDMayo ClinicMayo Clinic
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DisclosuresDisclosures
None
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Adrenal CaseAdrenal Case
HPIHPI• 55 y/o female55 y/o female• 1 yr ago: L mast, – SLN for 3.5 cm infiltrating 1 yr ago: L mast, – SLN for 3.5 cm infiltrating
ductal carcinoma; ER, PR +ductal carcinoma; ER, PR +• 4 cycles AC chemotherapy; Tam4 cycles AC chemotherapy; Tam• Energy and WBC (2,600) did not return to nlEnergy and WBC (2,600) did not return to nl• Home MD further investigatedHome MD further investigated
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Adrenal: Patient Eval Adrenal: Patient Eval
Meds: TamoxifenMeds: TamoxifenPSH: 2 back operations; appyPSH: 2 back operations; appyPMH: Well except breast CaPMH: Well except breast CaExam: VS nl Wt 120 lbsExam: VS nl Wt 120 lbs• Chest: mast site negChest: mast site neg• Nodes: all negNodes: all neg• Abd, pelvis: negAbd, pelvis: neg
Labs: Lytes, glu, Cr, LFTs, CXR nlLabs: Lytes, glu, Cr, LFTs, CXR nl
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CT ImagingCT Imaging
CT: 6 cm mass replacing R adrenal. L adrenal nl; no hepatic masses; no abdominal lymphadenopathy
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Adrenal Mass: EvaluationAdrenal Mass: Evaluation
What further testing?What further testing?• Serum, urine aldosteroneSerum, urine aldosterone• AM, PM cortisolAM, PM cortisol• 24-hr urine 24-hr urine ““mets and catsmets and cats””• 1 mg overnight dex suppression1 mg overnight dex suppression• CT-directed biopsyCT-directed biopsy• Bone scanBone scan• PET scanPET scan
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Adrenal Mass: TestingAdrenal Mass: Testing
TestTest• AldoAldo• CortisolCortisol• PheoPheo• Dex suppDex supp• BxBx
• Bone scanBone scan• PET scanPET scan
ResultsResults• Not DoneNot Done• NormalNormal• NormalNormal• Not DoneNot Done• Small sample: positive for Small sample: positive for
malignancy, c/w breast camalignancy, c/w breast ca• No metsNo mets
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PET ScanPET Scan
No abnormal uptake
except a large mass in the
right adrenal gland,
consistent with
malignancy.
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Adrenal Mass: ManagementAdrenal Mass: Management
Options?Options?
1.1. Switch to different br ca drugSwitch to different br ca drug
2.2. Laparoscopic adrenalectomyLaparoscopic adrenalectomy
3.3. Open anterior adrenalectomyOpen anterior adrenalectomy
4.4. Right adrenal radiationRight adrenal radiation
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Adrenal Cancer: Adrenal Cancer: TreatmentTreatment
SurgicalSurgical
• Open anterior right total adrenalectomyOpen anterior right total adrenalectomy
PathologyPathology
• 7.5 cm, 115 gm adrenocortical carcinoma; 7.5 cm, 115 gm adrenocortical carcinoma; no extra-adrenal invasionno extra-adrenal invasion
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Adrenal IncidentalomaAdrenal IncidentalomaObjectivesObjectives
Following this presentation, you shouldFollowing this presentation, you should• Understand the definition and frequency Understand the definition and frequency
of adrenal incidentaloma of adrenal incidentaloma • Be prepared to evaluate an incidentaloma Be prepared to evaluate an incidentaloma
and make management recommendationsand make management recommendations• Understand the pitfalls and nuances of Understand the pitfalls and nuances of
the management algorithmthe management algorithm
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Adrenal IncidentalomaAdrenal IncidentalomaDefinitionDefinition
Criteria1 cm diameter
• Well defined
Exclude
• Suspected hormonal hyperfunction
• Prior/concurrent malignancy
• Localized tumor symptoms/signs
• Constitutional symptoms of malignancy
Criteria1 cm diameter
• Well defined
Exclude
• Suspected hormonal hyperfunction
• Prior/concurrent malignancy
• Localized tumor symptoms/signs
• Constitutional symptoms of malignancy
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Adrenal IncidentalomaAdrenal IncidentalomaHistoricalHistorical
Autopsy series
• 1.4-15% incidence
• Average size 1 cm
• Frequency increases with age
Problem recognition
• 1982 Geelhoed, 20 patients
• 1983 Prinz, 9 patients
Autopsy series
• 1.4-15% incidence
• Average size 1 cm
• Frequency increases with age
Problem recognition
• 1982 Geelhoed, 20 patients
• 1983 Prinz, 9 patients
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Adrenal IncidentalomaAdrenal IncidentalomaNl Anatomy, Early CTNl Anatomy, Early CT
Nl adrenalsNl adrenals Incidentaloma, 1983Incidentaloma, 1983
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Adrenal IncidentalomaAdrenal IncidentalomaAge DependenceAge Dependence
0
1
2
3
4
5
6
7
8
Pat
ien
ts, %
0-9 10-19 20-29 30-39 40-49 50-59 60-69 70-79
Age, yrs
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Adrenal IncidentalomaAdrenal IncidentalomaAlgorithmAlgorithm
Adrenal tumorAdrenal tumor
ScreenScreen ExciseExciseObserveObserve
AssessmentAssessmentfor malignancyfor malignancy
Evaluation forEvaluation forhyperfunctionhyperfunction
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Adrenal IncidentalomaAdrenal IncidentalomaEndocrine HyperfunctionEndocrine Hyperfunction
Possible Occult Functioning TumorsPossible Occult Functioning Tumors
• PheochromocytomaPheochromocytoma
• AldosteronomaAldosteronoma
• CushingCushing’’s adenomas adenoma
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Adrenal IncidentalomaAdrenal IncidentalomaPheochromocytomaPheochromocytoma
IncidentalomasIncidentalomas• 5.1% prove to be pheochromocytomas5.1% prove to be pheochromocytomas• 7% pts with pheos have nl 24-hr urinary 7% pts with pheos have nl 24-hr urinary
fractionated fractionated catecholaminescatecholamines• 7% have nl 24-hr urinary total 7% have nl 24-hr urinary total
metanephrinesmetanephrines• 99%99% of of functioningfunctioning pheos have increased pheos have increased
levels of one or both of above levelslevels of one or both of above levels
24 hr urinary mets and cats
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Adrenal IncidentalomaAdrenal IncidentalomaPrimary AldosteronismPrimary Aldosteronism
ScreeningScreening
• Most frequent cause of 2º hypertensionMost frequent cause of 2º hypertension
• Morning Morning PAC/PRAPAC/PRA
– On any antihypertensive except On any antihypertensive except spironolactonespironolactone
– Ratio of Ratio of 20 and a PAC of 15 ng/dL is 20 and a PAC of 15 ng/dL is positivepositive
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Adrenal Incidentaloma Adrenal Incidentaloma Preclinical CushingPreclinical Cushing’’s Syndrome (PCSs Syndrome (PCS))
DefinitionDefinition
• Pts who lack typical signs/sxs of Pts who lack typical signs/sxs of
hypercortisolism having an incidentaloma hypercortisolism having an incidentaloma
with autonomous cortisol secretionwith autonomous cortisol secretion
• Pts may exhibit side effects of Pts may exhibit side effects of
endogenous cortisol secretionendogenous cortisol secretion
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Adrenal IncidentalomaAdrenal IncidentalomaFrequency--PCSFrequency--PCS
Study Group--Italian Soc EndocrinologyStudy Group--Italian Soc Endocrinology• 786 patients786 patients• 49 (6.2%) with PCS49 (6.2%) with PCS
Continuum of glucocorticoid autonomyContinuum of glucocorticoid autonomy• Most adrenal adenomas have functional Most adrenal adenomas have functional
autonomyautonomy• PCS may develop over extended period (1.7-10 PCS may develop over extended period (1.7-10
yrs) yrs) repeat hormonal screening at 1-yr F/Urepeat hormonal screening at 1-yr F/U
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Adrenal IncidentalomaAdrenal IncidentalomaEvaluation--PCSEvaluation--PCS
Laboratory CriteriaLaboratory Criteria
• 1-mg dex suppression test1-mg dex suppression test
– 8 AM cortisol level > 5 8 AM cortisol level > 5 g/dLg/dL
– Confirm with 2-day low-dose dex Confirm with 2-day low-dose dex
suppression testsuppression test
• ACTH assays--most not sensitive enoughACTH assays--most not sensitive enough
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Adrenal IncidentalomaAdrenal IncidentalomaAdvantages vs DisadvantagesAdvantages vs Disadvantages
•Op mort/morb•XS surg for
benign lesions
•Op mort/morb•XS surg for
benign lesions
Risk of malignancy
Risk of malignancy
ExcisionExcision ObservationObservation
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Adrenal IncidentalomaAdrenal IncidentalomaMalignant PotentialMalignant Potential
Potential Distinguishing CriteriaPotential Distinguishing Criteria
• Mass SizeMass Size
• Imaging phenotypeImaging phenotype
• Image-guided needle biopsyImage-guided needle biopsy
–MetastasisMetastasis
– InfectionInfection
• Iodocholesterol scintigraphyIodocholesterol scintigraphy
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Adrenal IncidentalomaAdrenal IncidentalomaImaging PhenotypeImaging Phenotype
CT characteristicsCT characteristics• Density--Hounsfield scaleDensity--Hounsfield scale• 0 = water 1,000 = bone -1,000 = air0 = water 1,000 = bone -1,000 = air• -20 to -150 = adipose-20 to -150 = adipose• 20 to 50 = kidney20 to 50 = kidney• If adrenal is < 10, nearly 100% benign If adrenal is < 10, nearly 100% benign
adenomaadenoma• IV contrast:IV contrast:• Modest enhancementModest enhancement• Rapid washoutRapid washout
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Adrenal IncidentalomaAdrenal IncidentalomaExampleExample
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Adrenal IncidentalomaAdrenal IncidentalomaExampleExample
Precontrast Postcontrast
Report: 2.8 cm, precontrast 22-28 HU, immediate Report: 2.8 cm, precontrast 22-28 HU, immediate postcontrast 32-38 HU; Delayed 56-60 HU--postcontrast 32-38 HU; Delayed 56-60 HU--NotNot diagnostic for adenoma (Pathology: adrenal adenoma)diagnostic for adenoma (Pathology: adrenal adenoma)
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Adrenal IncidentalomaAdrenal Incidentaloma Imaging PhenotypeImaging Phenotype
CT Characteristics (contCT Characteristics (cont’’d)d)• ShapeShape–Smooth, round/oval vs IrregularSmooth, round/oval vs Irregular
• TextureTexture–Homogeneous vs heterogeneousHomogeneous vs heterogeneous
• LateralityLaterality–Uni- vs bilateralUni- vs bilateral
• OtherOther–Hemorrhage, necrosis, calcificationsHemorrhage, necrosis, calcifications
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Adrenal IncidentalomaAdrenal IncidentalomaCharacteristicsCharacteristics
AdenomaAdenoma• Size--small, typically Size--small, typically 3 cm 3 cm• Shape--round to oval, smooth marginsShape--round to oval, smooth margins• Texture--homogeneous, low densityTexture--homogeneous, low density• Laterality--solitary, unilateralLaterality--solitary, unilateral• Contrast enhancement--limitedContrast enhancement--limited• MR imaging--isointense to liver on T2-weighted imageMR imaging--isointense to liver on T2-weighted image• Necrosis, hemorrhage, CaNecrosis, hemorrhage, Ca2+2+--rare--rare• Growth--usually stable, very slow growthGrowth--usually stable, very slow growth
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Adrenal IncidentalomaAdrenal IncidentalomaCharacteristicsCharacteristics
MRI: MRI: Typical appearance of Typical appearance of ““in phasein phase”” and and ““out of out of phasephase”” cuts with signal drop cuts with signal drop out typical for benign out typical for benign adenomaadenoma
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Adrenal DisordersAdrenal DisordersBenign Nonfunctioning AdenomaBenign Nonfunctioning Adenoma
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Adrenal IncidentalomaAdrenal Incidentaloma Imaging PhenotypeImaging Phenotype
Imaging: >25 HU precontrast; enhancing rimImaging: >25 HU precontrast; enhancing rimSurgical Dx: Surgical Dx: pheochromocytomapheochromocytoma
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Adrenal IncidentalomaAdrenal IncidentalomaCharacteristicsCharacteristics
PheochromocytomaPheochromocytoma• Size--large, typically > 3 cmSize--large, typically > 3 cm• Shape--round, oval, clear marginsShape--round, oval, clear margins• Texture--inhomogeneous with cystic areasTexture--inhomogeneous with cystic areas• Laterality--solitary, unilateralLaterality--solitary, unilateral• Contrast enhancement--vascular, markedContrast enhancement--vascular, marked• MR--markedly hyperintense on T2MR--markedly hyperintense on T2• Necrosis, hemorrhage, CaNecrosis, hemorrhage, Ca2+2+--hemorrhage and cystic --hemorrhage and cystic
necrosis commonnecrosis common• Growth--usually slowGrowth--usually slow
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Adrenal IncidentalomaAdrenal IncidentalomaPheochromocytomaPheochromocytoma
““IncidentalIncidental”” pheo pheo Typical pheoTypical pheo
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Adrenal IncidentalomaAdrenal IncidentalomaCharacteristicsCharacteristics
Adrenocortical carcinomaAdrenocortical carcinoma• Size--large, typically > 4 cmSize--large, typically > 4 cm• Shape--irregular, unclear marginsShape--irregular, unclear margins• Texture--inhomogeneous, mixed densitiesTexture--inhomogeneous, mixed densities• Laterality--solitary, unilateralLaterality--solitary, unilateral• Contrast enhancement--vascular, markedContrast enhancement--vascular, marked• MR--hyperintense on T2MR--hyperintense on T2• Necrosis, hemorrhage, CaNecrosis, hemorrhage, Ca2+2+--common--common• Growth--rapidGrowth--rapid
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Adrenal IncidentalomaAdrenal IncidentalomaExamplesExamples
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Adrenal IncidentalomaAdrenal IncidentalomaCharacteristicsCharacteristics
MetastasisMetastasis• Size--variable, frequently < 3 cmSize--variable, frequently < 3 cm• Shape--oval to irregular, unclear marginsShape--oval to irregular, unclear margins• Texture--inhomogeneousTexture--inhomogeneous• Laterality--often bilateralLaterality--often bilateral• Contrast enhancement--vascular, enhancement tumor Contrast enhancement--vascular, enhancement tumor
rimrim• MR--hyperintense on T2MR--hyperintense on T2• Hemorrhage/cystic necrotic areas commonHemorrhage/cystic necrotic areas common• Growth--usually slowGrowth--usually slow
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Adrenal IncidentalomaAdrenal Incidentaloma Imaging PhenotypeImaging Phenotype
Surgical Dx:Surgical Dx:Metastatic colon Metastatic colon carcinomacarcinoma
CharacteristicsCharacteristics•HeterogeneousHeterogeneous•Irregular borderIrregular border•Enhancing rimEnhancing rim
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Adrenal IncidentalomaAdrenal IncidentalomaUnusual TumorsUnusual Tumors
Tb, AddisonTb, Addison’’ssMalignant fibrous Malignant fibrous
histiocytomahistiocytoma
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Adrenal IncidentalomaAdrenal IncidentalomaUnusual TumorsUnusual Tumors
Cystic large cell lymphoma of adrenalCystic large cell lymphoma of adrenal
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Adrenal IncidentalomaAdrenal IncidentalomaUnnecessary SurgeryUnnecessary Surgery
1 2 3 4 5 6 7 8 9 10 110
10
20
30
40
%%
Size of tumor (cm)Size of tumor (cm)
(115) (115)
(65)
(22)(12) (4)
(3) (1)
32%
6%
12%
Malignant tumors
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Adrenal IncidentalomaAdrenal IncidentalomaAlgorithmAlgorithm
Based on observations:Based on observations: 10% incidentalomas hyperfunctional, 10% incidentalomas hyperfunctional,
autonomousautonomous
• < 5% adrenocortical carcinomas< 5% adrenocortical carcinomas
• 95% adrenocortical cancers > 4 cm95% adrenocortical cancers > 4 cm
• 95% cortical adenomas < 5 cm95% cortical adenomas < 5 cm
• Imaging phenotype very helpfulImaging phenotype very helpful
• FNA rarely indicatedFNA rarely indicated
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Adrenal IncidentalomaAdrenal IncidentalomaAlgorithmAlgorithm
H & P24-hr urine mets, cats1-mg overnight DST
If BP: PAC/PRA
Nonfunctional,< 4 cm
Functioning mass,> 4 cm
Surgicalresection
Repeat imagingNo change--observe
If > 1cmIf > 1cm
sizesize
Imaging phenotype
Benign Suspicious
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Thank
you