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A 48 year-old male patient with refractory hypertension on four antihypertensive medications is found to have Cushings syndrome.
Review of his medications reveals no exogenous glucocorticoid
administration. Which lab result would confirm that this patient is a
candidate for laparoscopic adrenalectomy?
Suppression of cortisol with low-dose dexamethasone
High Cortisol
Low ACTH
Mild increase of cortisol in response to administration of corticotrophin
releasing hormone
High ACTH
Discussion: Work-up of the patient with Cushings syndrome requires
identification of the source of the excess cortisol production. One would
expect Cushings syndrome of any etiology to have an elevated cortisol
level. In a normal patient, cortisol levels would be suppressed by
administration of low-dose dexamethasone. A high ACTH is indicative
of a pituitary source, also known as Cushings disease. A low ACTH
level is indicative of a non-pituitary source; in this case making an
adrenal source the most likely etiology.
vein. Both adrenal glands have arterial blood supply via direct
branches from the aorta, renal arteries, and phrenic arteries.
48yearoldmalesmokerwith30packyearhistorywithnoadditionalsignificantpast
medicalhistoryisbroughttotheemergencyroomafterafallfromscaffoldingat
work.Onarrival,hewasnormotensiveat110/60,P:80,withtemperatureof37.On
physicalexam,hecomplainedoflegpainontheleftandtheremainderofhisphysical
examincludingneurologicalevaluationwasnormal.HisPAandlateralchestxray
revealednoevidenceofpneumothorax.HeunderwentCTscanoftheabdomenand
pelviswhichrevealeda6.2cmleftadrenalnodule.Laboratoryresultsdidnot
demonstratefunctionality.Whatisthenextstepinmanagement?
Openadrenalectomy
RuleoutlungcancerbyobtainingCTscan
Observation
Laparascopicadrenalectomy
CTguidedbiopsyofadrenalmass
ThisisapatientwithhistoryofsmokingwithanincidentallyfoundadrenalmassonCT
Scan.Theriskofcancerincreaseswithincreasingsize.Atthispointwithlesionmore
than6cm,itshouldberecommendedthatheundergolaparoscopicadrenalectomy.
Althoughnotclearlylinked,thereisanassociationbetweensmokinganddevelopmentof
adrenalmalignancy.
A 62 year old male underwent a CT scan for evaluation for right lower
quadrant pain to rule out appendicitis. His medical history is
significant for hypertension, hypercholesterolemia, and recent MI three
months ago that required cardiac catheterization and placement of
drug eluting stent. His CT scan was unremarkable for appendicitis but
incidentally he was found to have 2.5 cm adrenal nodule on the right
side. Further work up revealed normal serum and urine catecholamine
levels, and a plasma aldosterone/renin ratio of 31. His current
medication includes a beta-blocker, ACE-inhibitor, and calcium channel
blocker. On physical exam, he was normotensive at 120/80, P: 65 and
otherwise in good health. What would you advise this patient at this
point?
Observe the mass as it is a small incidentaloma that is hormonally
inactive and the there is no benefit to excision
Open adrenalectomy for functional mass
Observe the mass as the risk outweighs the benefit at this point
Laparascopic adrenalectomy for non-functional adrenal mass
Laparascopic adrenalectomy for functional adrenal mass
This is a functional aldosteronoma with plasma aldosterone/renin ratio
over 30. In a normal circumstance, where there is a proven functional
adrenal nodule, the patient would undergo adrelenalectomy. In this
patient with recent MI with drug eluting stent, he will need to be on
anticoagulants for more than three months. Furthermore, his blood
pressure is currently is well controlled with his anti-hypertensive
medications. The risk of this patient undergoing the operation
outweighs the benefit at this point. He will be better served with
observation until he is medically optimized or he becomes more
symptomatic.
A38yearoldfemalepresentswithfrequentboutsofheadaches.Shehasbeenonthreedifferentanti
hypertensivemedicationssincetheageof36.Despitemaximizingthedosageoftheantihypertensive
medications,hersystolicbloodpressurecontinuestoremaininthe160s.SheiscurrentlytakinganACE
Inhibitor,aBBlocker,andaCalciumchannelblocker.ShehadCTscanwhenshewas30yearsoldafter
anautomobileaccidentandwastoldthatshemayhavesmallnodularmassonherrightadrenalgland.
Onphysicalexam,sheiswellappearingwithnopalpablemassonabdominalexam.Hertemperatureis
98.7,Bloodpressureis160/60,HRof80.Herfundoscopicexaminationisunremarkable.Routineblood
testsshowedthefollowing:
BMP
Sodium144meq/L
Sodium144meq/L
Pottasium2.9meq/L
Chloride103meq/L
Bicarbonate30meq/L
Creatinine1.1mg/dL
UrinalysisNormal
UrineSodiumlow
UrinePotassiumhigh
Whatisthenextbeststepinmanagementforthispatient?
CTscanoftheabdomenandpelvis
Measurementofplasmametanephrineandnormetanephrinelevels
Dexamethasonesuppressiontest
RenalAngiography
Plasmarenin/aldosteronelevel
Youranswerisnotcorrect
Thispatienthasanaldosteronoma.Thedifferentialdiagnosisforpatientwhopresentswithuncontrolled
hypertensionincludesPheochromocytoma,Aldosteronoma,CushingsDisease,andRenalHypertension.
Thepatientabovepresentedwithuncontrolledhypertensionaccompaniedbylowserumpotassiumlevel
andslightlyelevatedserumsodiumlevel.Furthermore,thispatientalsohaselevatedurinepotassiumwhich
isconsistentwiththediagnosisofaldosteronoma.
A55yearoldmanisevaluatedfora10monthhistoryofrecurrentepisodesofsweating,palpitations,and
headaches.Heotherwisehasanunremarkablemedicalhistory.HeiscurrentlytakingAtenololand
Lisinopriltocontrolhisbloodpressure.Physicalexamrevealedaveryanxiousappearingmanwithsweaty
palmsandneckexamsignificantfornormalthyroidglands.Hisvitalsignsincludeatemperatureof37.8C,
bloodpressureof162/95,pulserateof90bpmandrespiratoryrateof16breathsperminute.
Laboratorystudiesrevealedelevatedspotplasmametanephrinelevels.HesubsequentlyundergoesCTscan
oftheabdomenandpelvisdemonstratesa5mmnodulewithintherightadrenal.MRIoftheabdomen
revealsasimilarfinding,otherwisenegative.Whatisthenextbeststepinthemanagementofthispatient?
PETScan
LaparoscopicrightAdrenalectomy
VenographywithvenoussamplingbilateralAdrenals
MIBGScan
Ultrasoundoftheabdomen
Youranswerisnotcorrect
Thispatienthassignsandsymptomsofpheochromocytoma.Biochemicallyitwasshownthathehas
elevatedmetanephrinelevelsconsistentwithdiagnosisofpheochromocytoma.However,neithertheCT
scanoftheabdomennortheMRIoftheabdomencouldbeconsidereddefinitivelocalizationasthe?mass
seenisverysmallandcouldbeanincidentaladenomaorevenafalsepositiveread(sincesosmall).The
nexttesttolookforadrenalmassistheMIBGscan.Thisisatestthatutilizesaradioactiveisotopethatis
selectivelytakenupbytissuesthatsecretecatecholamines.MIBGscanisusuallyreservedforpatients
wheretheCTscanfindingisequivocalortodiagnoseextraadrenalpheochromocytoma.Inthiscaseit
wouldnotbehelpfulasevenifthis5mmmassisthelesion,itistoosmalltopickupbynuclearscanand
alsoitiswithintheadrenal,sonothelpfulasadrenalswillhaveanormalphysiologicuptakebyMIBG
(thatiswhyitishelpfultoassessanextraadrenalmass).Venographywithvenoussamplingofbilateral
adrenalglandsmaybeusefulwhendealingwithbilateraladrenalmassesandforplanningsurgical
resectiontoseewhetheranoduleisbiochemicallyactive.Thedownsidetothetestisthatitisinvasiveand
shouldbereservedafternoninvasivetestinghasbeenattempted.
A22yearoldfemalewithclinicalsuspicionforhypercortisolismisseenbytheendocrinologistforfollow
upafterobtaininglaboratorywork.InadditiontoherCushinoidfeatures,allofthelabresultsareconsistent
withhypercortisolism.Sheisnowinyourofficetodiscusstheresultsandthenextstepin
management.Herlaboratoryresultsareasfollows.:
Adrenocorticotropichormone:low
Urinefreecortisol:Elevated
Cortisol(8AM)
After1mgofdexamethasonethenightbefore:Elevated
After8mgofdexamethasonethenightbefore:Elevated
Whattestdoyouordernext?
Serum/urine catecholamine
CT scan of the head
CT Scan of the abdomen
MRI of the head
Cosyntropin stimulation test
Youransweriscorrect
This is a patient with suspected hypercortisolism. With low ACTH and cortisol levels that are not
suppressed after high and low dose dexamethasone suppression test, one of the suspicions is
that the patient has an ectopic adrenal nodule that is responsible for the results shown above. On
the contrary, patients who have pituitary adenomas resulting in Cushings will have elevated
ACTH, elevated urine free cortisol, and high dexamethasone suppression test will partially
depress the cortisol level. Thus, in this patient, one of the areas to evaluate is the adrenal gland
by obtaining CT Scan of the abdomen.