Adrenal IncidentalomaAdrenal Incidentaloma

Adrenal IncidentalomaAdrenal Incidentaloma

• Adrenal mass detected during abdominal imaging done for other reasons.

• Autopsy series - adrenal masses in 1.4 - 9%

• Noted in 5% of patients who undergo CT scans

The Adrenal Gland

The Adrenal Gland

Differential Diagnosis of Adrenal Incidentalomas

• Cortical Adenoma

• Cysts

• Hemorrhage

• Myelipoma

• Pheochromcytoma

• Gnaglioneuroma

• Adrenal Carcinoma

• Metastatic Cancer

• Granulomatosis

• Tuberculosis

• histoplasmosis,

• blastomycosis

• Abscess

• Amyloidosis

• Fibroma

Two main questions whenTwo main questions whenworking up an Incidentalomaworking up an Incidentaloma

Is it a Functioning Mass?

Is it a carcinoma?

Is it a Functioning Is it a Functioning Mass?Mass?

•Aldosterone•Cortisol•DHEAS•Pheochromocytoma

Patient presents with hypertension and hypokalemia. Found to have a 1.3 cm left adrenal mass.

Further workup revealed a high serum aldosterone and a low serum renin.

What is this consistent with ?

Aldosterone secreting adrenal adenoma (Conn's syndrome).

•Symptoms are often non specific

•Hypokalemia •weakness, cramps, polyuria

•Hypertension•may be severe and refractory to treatment

•Metabolic alkalosis

HyperaldosteronismHyperaldosteronism

Primary Primary hyperaldosteronismhyperaldosteronism

Subtypes of primary AldosteronismSubtypes of primary Aldosteronism

•Aldosterone producing adenomaAldosterone producing adenoma•Idiopathic hyperaldosteronismIdiopathic hyperaldosteronism•Primary adrenal hyperplasiaPrimary adrenal hyperplasia•Aldosterone producing adrenocortical carcinomaAldosterone producing adrenocortical carcinoma•Aldosterone producing ovarian tumorAldosterone producing ovarian tumor•Familial hyperaldosteronismFamilial hyperaldosteronism

A lg o rith m fo r H yp e ra ld o s te ro n ism

D iscon tinu e sp iron o lac to ne

sp on ta ne o us hyp oka lem ia D iu re tic in d uce d hyp o ka le m ia

C lin ica l su sp ic io n o f P A

K + > 4 .0P A u n like ly

K + < 4 .0

D iscon tin ue d iu re tic s a n d re p lace K +R e pe a t K + 2 w ee ks la te r o n a dd e d sa lt d ie t

P A co n firm ed

E le va ted a ld o s te ron e exc re tion

D iscon tin e in te rfe rin g m e dsK C L su pp le m e n ts

H ig h sod iu m d ie t fo r 3 d a ys a nd ob ta in2 4 hr u rin e fo r a ld os te ro ne

S c rfee n ing p os it ive if :P A C /P R A ra tio > 20

P A C > 2 0n g /d l

Random PRA and PAC

Random PRA and PAC

PA = Primary PA = Primary AldosteronismAldosteronism

Primary AldsteronismPrimary Aldsteronism

•If adrenal mass is > 1 cm proceed with adrenalectomyIf adrenal mass is > 1 cm proceed with adrenalectomy

•If mass is < 1 cm, then recommend adrenal vein samplingIf mass is < 1 cm, then recommend adrenal vein sampling

•if lateralises then adrenalectomyif lateralises then adrenalectomy

•if not - ? Idiopathic hyperaldosteronismif not - ? Idiopathic hyperaldosteronism

Is it a functioning mass?

CortisolCortisol

What is Cushing’s Syndrome?What is Cushing’s Syndrome?

List some features of the syndrome !List some features of the syndrome !

HypercortisolismHypercortisolismACTH dependentACTH dependent

•pituitary cushing’spituitary cushing’s•ectopic ACTHectopic ACTH

ACTH independent ( 20% )ACTH independent ( 20% )•adrenal adenoma/carcinoma/hyperplasiaadrenal adenoma/carcinoma/hyperplasia•iatrogeniciatrogenic

Facial fullnessFacial fullness

Truncal obesityTruncal obesity

Skin atrophy/bruisingSkin atrophy/bruising

Hirsutism/AcneHirsutism/Acne

Glucose intoleranceGlucose intolerance

Gonadal dysfunctionGonadal dysfunction

Muscle weaknessMuscle weakness

EdemaEdema

Cushing’s SyndromeCushing’s Syndrome

•Really Subclinical Cushing’s Really Subclinical Cushing’s

•Rarely progresses to overt Cushing’s Rarely progresses to overt Cushing’s

•Found in mean rate of adrenal incidentalomas 8%Found in mean rate of adrenal incidentalomas 8%

•Incidentalomas < 1cm do not need w/u for Cushing’s Incidentalomas < 1cm do not need w/u for Cushing’s •since hypercortisolism extremely raresince hypercortisolism extremely rare

Low Dose Dexamethasone Suppression TestLow Dose Dexamethasone Suppression Test

Day 1 Day 1 •at 0900 take fasting blood cortisol at 0900 take fasting blood cortisol •Immediately inject 0.5mg DexamethasoneImmediately inject 0.5mg Dexamethasone•Give further 0.5mg at 1500,2100 and 0300 hrsGive further 0.5mg at 1500,2100 and 0300 hrs

Day 2Day 2•at 0900 take fasting blood cortisol at 0900 take fasting blood cortisol •Immediately inject 0.5mg DexamethasoneImmediately inject 0.5mg Dexamethasone•Give further 0.5mg at 1500,2100 and 0300 hrsGive further 0.5mg at 1500,2100 and 0300 hrs

Day 3Day 3•at 0900 take fasting blood cortisol at 0900 take fasting blood cortisol

The High Dose Test is the same using 2mgThe High Dose Test is the same using 2mg

Cushing’s Syndrome caused by adrenal tumorCushing’s Syndrome caused by adrenal tumor•low dose: no changelow dose: no change•high dose:no changehigh dose:no change

Cushing’s DiseaseCushing’s Disease•low dose: no changelow dose: no change•high dose: normal suppressionhigh dose: normal suppression

Cushing’s Syndrome caused by ectopic ACTHCushing’s Syndrome caused by ectopic ACTH•low dose: no changelow dose: no change•high dose:no changehigh dose:no change

Is it a functioning mass ?Is it a functioning mass ?

DHEAS - DHEAS - Dehydroepiandrosterone sulfate

secreted directly and almost exclusively by the adrenal

Signs of Virilization in women !Signs of Virilization in women !

•hirsutism •temporal balding•voice changes•clitoral enlargement•acne•increased muscle mass •decreased breast size •amenorrhea

1.The medulla is composed of ____________ cells derived

from the_____________.

chromaffin

neural crest.

2.Most extra-adrenal pheochromocytomas are located at the

___________________________Organ of Zuckerkandl

3.Patients with pheochromocytomas in ____________wall may

present with __________ loss of consciousness

due to catecholamine release.

the bladder

post-voiding

Before we proceed…….Before we proceed…….

PheochromocytomaPheochromocytomaSecrete both epi and norepinephrineSecrete both epi and norepinephrine< 0.1% of hypertensive patients< 0.1% of hypertensive patients85 - 90% - Adrenal Medulla85 - 90% - Adrenal MedullaMEN - multiple endocrine neoplasiaMEN - multiple endocrine neoplasia•Type II A - medullary CA, parathyroidType II A - medullary CA, parathyroid•Type II B - medullary CA, neuromas, Type II B - medullary CA, neuromas, marfanoidmarfanoid

malignantmalignant

bilateral

extra-adrenal.

Familial

sporadic

Rule of 10

Complications of pheochromocytoma crisisshockDICseizuresrhabdomyolysisacute renal failuredeath.

Urinary metanephrines above 1.2 mg/day (6.5 µmol/day)

false-positive results can very rarely occur

•labetalol

•buspirone •caffeine

Pheochromocytoma Work upPheochromocytoma Work upLabs:Labs:ElectrolytesElectrolytes24 hr urine metanephrines24 hr urine metanephrinesPlasma catecholaminesPlasma catecholamines

Biochemical test Sensitivity Specificity

sMetanephrine 99% 89%

sCatecholamine 85% 80%

uMetanephrine 76% 94%

uCatecholamine 83% 88%

uVMA 63% 94%

Ann Int Med (2001)134:315-329

CT and MRI have higher sensitivity than CT and MRI have higher sensitivity than 131131I-MIBGI-MIBG

However, However, 131131I-MIBG uptake is more specificI-MIBG uptake is more specific

Some authors prefer to use MIBG as the initial screening modalitySome authors prefer to use MIBG as the initial screening modality

because it enables whole-body imagingbecause it enables whole-body imaging

makes it useful for detection of extra-adrenal tumors and metastatic makes it useful for detection of extra-adrenal tumors and metastatic deposits. deposits.

the false-negative rate of MIBG scintigraphy is 10%.the false-negative rate of MIBG scintigraphy is 10%.

CT/MRI still doneCT/MRI still done

CT - quick/relatively inexpensive but not specific enough to CT - quick/relatively inexpensive but not specific enough to distinguish massesdistinguish masses

hypertensive crisis after the injection of contrast material - reportedhypertensive crisis after the injection of contrast material - reported

MRI - more specific than CT, but some patients cannot tolerate MRIMRI - more specific than CT, but some patients cannot tolerate MRI.

ImagingImaging for for PheochromocytomaPheochromocytoma

Ph

eoch

rom

ocyto

ma

Ph

eoch

rom

ocyto

ma

Is it Malignant? Is it Malignant?

Pt’s with known malignancyPt’s with known malignancy•8 -38% pts with known malignancy 8 -38% pts with known malignancy •have adrenal mets at autopsyhave adrenal mets at autopsy•Most common primaryMost common primary

•breastbreast•lunglung•kidneykidney•melanomamelanoma•lympomalympoma

•CT guided FNA may be of valueCT guided FNA may be of value

•Adrenocortical carcinoma - 0.5/million - incidenceAdrenocortical carcinoma - 0.5/million - incidence

•5 year survival5 year survival•localized disease:70%localized disease:70%•metastases:5.3%metastases:5.3%

Imaging: favoring benignImaging: favoring benign

Small sizeSmall sizeRound regular shapeRound regular shapehomogeneityhomogeneitylow density on CTlow density on CTlow T2 weighted signallow T2 weighted signalhigh lipid contenthigh lipid content

Imaging: favoring malignantImaging: favoring malignant

Large size ( >5cm )Large size ( >5cm )irregular shapeirregular shapeheterogeneityheterogeneityhigh density on CThigh density on CThigh T2 weighted signalhigh T2 weighted signallow lipid contentlow lipid contentdemonstratable growthdemonstratable growth

SIZESIZE - it does matter !- it does matter !

The probability of malignancy increases with sizeThe probability of malignancy increases with sizeAt a cut off of 4cm, the mal/benign ratio - 8:1At a cut off of 4cm, the mal/benign ratio - 8:1Recommendations - remove all lesions > 4 cmRecommendations - remove all lesions > 4 cm

It is nonfunctioning and less than 4cm , now what?

Adrenal IncidentalomaAdrenal Incidentaloma

Im a ge d ia g no s tic if kno w n m e ts

2 4 h r m e tan ep h rin esP A /P R A , K +,

D H E A S , L o w d o se D e x,

F N A if su sp ec t m e ts

R e v ie w F ilm s

N o ch an geC o n tin u e fo llow up

S iz e inc re a se > 1 cmP A /P R A , K +,

D H E A S , L o w d o se D e x,

N o n fun c tion in g < 4 cmre pe a t im ag e in 3 an d 1 2 m on ths

A d ren a le cto m y

H o rm on a l h yp e rsec re tiono r m a ss > 5 cm

WATCH ADRENALECTOMY