Adrenal gland short note by S.

Wichien (SNG KKU)

Embryo Adrenal cortex -outer layer -80-90% of gland -mesoderm on adrenogenital ridge -ectopic adrenocor--ovary,sperm cord 3 zone 1.zona 2.zona 3.zona (GFR) glomerulosa--mineralo,aldos fasiculata--glucocorticoid reticularis--androgen Cushing synd -cushing dz=pituitary tumor -f:m=8:1, most=sporadic Hypercortisolism Exogenous steroid--most common Endogenous steroid 1.ACTH dependent 70% -pitu.adenoma -ectopic ACTH -ectopic CRH 2.ACTH independent 20-30% -adrenal adenoma -adrenal ca -adrenal hyperplasia 3.other -pseudo-cushing synd :alcohol,preg,CKD,stress Ectopic ACTH -SCLC,MTC,pancreatic islet cell, phaeochromo,carcinoid of lung Confirm test 1.low dose DST -dexa 1mg 11.00 pm -cortisol level 8.00 am -test +ve if <1.8g/dL Hi suspicions but test -ve Classic low dose DST -0.5 mg q 6 hr * 8 dose 2.urine 24hr free cortisol -r/o if <100 g/dL 3.11.00 PM salivary cortisol Source 1.plasma ACTH -normal 10-100 pg/mL ->1000 suggest ectopic ACTH 2.hi dose DST -2 mg q 6 hr *8 dose -24 hr urine cortisol over 2nd day -fail suppress 50%--dx ectopic ACTH A.dec ACTH+lack sup--adrenal B.inc ACTH+sup--pitu source C.inc ACTH+lack sup--ectopic ACTH Tx -lap.adrenalectomy--Tx of choice -open--large>6cm, suspect ca need pre-post op steroid due to suppress contralateral side

Adrenal medulla -inner layer -10-20% of gland -neural crest migration :para-aortic, paravertebral area :medial aspect of cortex -extra-adrenal neural tissue :organ of Zuckerkandl (lt of aortic bifurcate near IMA) :neck,bladder,pra-aortic -epi+norepi Anatomy -sup-medial to KN -11 th rib -5*3*1 cm -4-5 gm Artery -sup adrenal<--inf phrenic a -mid adrenal<--aorta -inf adrenal<--renal a Vein -single,major adrenal v -rt adrenal-->IVC -lt adrenal (+inf phrenic v)-->lt renal v

Adrenal gland short note by S.Wichien (SNG KKU)

Adrenal insuff Etiology 1--adrenal dz -autoimmune -TB,fungus,HIV,CMV -spon.hmg--meningococcal sepsis (waterhouse Friderichsen synd) -metastasis -infiltrative--amyloid,hemochromo -CAH -drugs--ketoco,mitotane,metyrapone 2--def ACTH -exo.steroid -bilat.adrenalectomy -pitu,hypothalamic tumor -pitu hmg--Sheehan synd Dx ACTH stimulation test -ACTH 250 mcg iv -cortisol level at 0,30,60 m -peak cortisol <20 g/d = dx ACTH level 1 - inc 2 - dec CAH -def/absence enz of steroidogenesis -most=21 hydroxylase (CYP21A2) :proges--CYP21A2--aldosterone :proges--17HP--CYP21A2--cortisol -def glucocorticoid+aldosterone -inc adrenal androgen :17HP--aldosterone inc -ACTH level -coticosteroid precursor--17-HP -adrenal androgen Comp def of 21 hydroxylase at birth -virilizing feature -diarrhea,hyponatremia,hyperk -hyperpigmentation Partial def 21 hydroxylase -virilizing feature Tx -med Tx--cortisol+aldos replacement -alter.Tx--bilat.lap adrenalectomy Sex H excess Virilze tumor -tumor secrete androgen -virilizing synd -hirsutism,amen,infertile,mascularize -male--dx difficult --advance stage -child--premature develop Dx -androgen level--serum DHEA -urine 17-ketosteroid Fiminize tumor -less common -male--gyneco,impoten,testis atrophy -female--irreg mense,AUB,precocious Dx -urine 17-ketosteroid -estrogen level Tx -adrenalectomy -metas dz--adrenolytic drug :mitotane,aminoglutethimid :ketoconazole

Adrenal gland short note by S.Wichien (SNG KKU)

Hyperaldosteronism 2hyperaldosteronism (stimulate RAAS from) -renal a stenosis -low flow state--CHF,cirrhosis 1hyperaldosteronism -30-50 yr -1% of HT -70% = solitary adenoma -30% = idiopathic bilat hyperplasia -1% = adrenocortical ca S&S -long standing HT -difficult control HT--multi drug -m weakness--hypoK -polydipsia,polyurea,nocturia Dx -k<3.2 K<3 despite k spaing drug (40%=normo k) -elevate plasma aldosterone suppress plasma renin :ratio 1 : 25-30 -saline loading test :5d Na diet or 2L of nss :can't suppress aldos :r/o if aldos<5 ng/dl or 24hr u<14 g CT/MRI if unilat.tumor -->adrenalectomy if bilat.abnormal/normal -->selective v cath or NP 59 scan 1.uni.inc aldos--adrenalectomy 2.bilat hyperfxn/fail localized--med Tx Selective venous cath -sen 95, spec 90% -cannulate adrenal v -aldos & cortisol level after ACTH -aldos:cortisal ratio (btw adrenal v) >4x = unilat.tumor Use in -ambiguous case -can't localized -bilat adrenal enlarge NP 59 scan -131I-6 iodomethyl noriodocholes -adrenoma = hot nodule -hyperplasia = bilat inc uptake Tx Pre-op -control HT :spironolactone,amiloride :nifedipine,ACEI -adequate k supplement--k > 3.5 Post-op -may transient hypaldostetone :require mineralocorti up to 3 m

Adrenal gland short note by S.Wichien (SNG KKU)

Phaeochromocytoma 10%tumor 1.bilateral 2.malignant -evident of invasion/metas 3.pediatric 4.extra-adrenal (fxn paraganglioma) -organ of Zuckerkandl -neck,medias,abdomen,pelvis 5.familial (MEN2a,2b) Asso -VHL,NF1,sturge-weber,TS,carney VHL -phaeochromocytoma (14%) -retinal angioma -CNS hemahioblastoma -renal ca -pancreatic cyst -epididymal cystadenoma Carney synd -gastric epitheloid -leiomyosarcoma -pulmo.chondroma -extra-adrenal paraganglioma S+S -headache+palpitate+diaphoresis -HT--most clinical sign Dx 24 hr urine catechcolamine urine catechcolamine -NE >2000 pg/ml -E >200 pg/ml urine metanephrine -sen 98% urine VMA -slightly less sen -false+ve--caffe,raw fruit,methyldopa plasma metanephrine -most reliable test clonidine suppression test -clonidine not suppress Phaeo -0.3 mg o --> dec cathe <500 chromogranin A -adrenal medulla -neuroendrocrine tumor Ix CT scan -no contrast--risk HT crisis -from diaphragm to aortic bifurcate (include organ zuckerkandl) MRI MIBG (Metaiodobenzylguanidine) Tx Pre-op 1.-block -1-3 wk -phenoxybenzamine,prazocin -orthostatic hypoT 2.-blocker -after adequate 1. -persistent tachycardia -propanolol 3.pre-op volume -p/o HT -sx--loss vasoconstrict Sx Open adrenalectomy -bilat tumor -extra adrenal lesion -metas lesion Lap.adrenalectomy -<5cm Hereditary phaeochromocytoma -multiple,bilat Tx -cortical sparing subtotal adrenalec

Adrenal gland short note by S.Wichien (SNG KKU)

Adrenocortical ca -bimodal age--child, 40-50 yr -major=sporadic -asso Li-Fraumeni synd (p53),MEN1, Beckwith-widemann,Carney complex S+S 50% nonfxn -enlarge abdo.mass 50% fxn -rapid onset cushing+ virilizing -30% cortisol 20% androgen 10% estrogen 2% aldosterone -35% multiple hormone Dx -elyte r/o hypok -urine cathecholamine r/o phaeo -DST,24 hr urine cortisal r/o cushing CT/MRI criteria for malignancy ->6cm -heterogeneity, irregular margin -adjacent lymphadenopathy -liver metastasis -signi lesion enhancement -T2 weight (adrenal:liver=1.2:2.8) -slow wash out of contrast Patho Gross -large tumor,area of hmg/necrosis Ddx from benign -nuclear gr 3,4 -mitotic rate >5/50 hpf -atypical mitosis -clear cell <25% of tumor -diffuse architecture -microscopic necrosis -invade venous,sinusoid,capsule Staging 1 T1N0M0 2 T2N0M0 3 T3N0M0, T1-2N1M0 4 T3-4N1M0 or M1 Tx -most predictor=adequate resect -en bloc resection -open adrenalectomy -sx debulking--isolate,recur dz Adjuvant Tx -mitotane--derivative of DDT -etoposide,cisplatin,doxo,paclitaxel

Adrenal gland short note by S.Wichien (SNG KKU)

Adrenal incidentaloma -0.4-4.4% on CT DDx Fxn lesion Benign -aldosteronoma -cortisol producing adenoma -sex steroid producing adenoma -phaeochromocytoma Malignant -adrenocortical ca -malignant phaeochromocytoma Non fxn Benign -cortical adenoma -myelolipoma -cyst -ganglioneuroma -hmg Malignant -metastasis (breast,lung,melano,RCC,lymphoma) -adenocortical ca Dx To identify pt that benefit from sx -fxn tumor -risk of ca Subclinical cushing -low dose DST -24 hr u cortisol Phaeochromocytoma -24 hr u catechco,metanephrine,VMA -plasma metanephrine Aldosteronoma -serum elyte -plasma aldosterone -plasma renin Risk of malignancy ->6cm (35%) -18 HU -inhomogeneous -irregular border -local invade,LN MRI (T2 weigth) -ca=mass to liver ratio = 1.2:2.8 -benign=mass to liver ratio <1.4 (low signal than liver) -Phaeo=mass to liver ratio >3 (extremely bright mass) Tx 1.is tumor fxn -yes--adrenalectomy -no--2 2.is it metastatic lesion (hx.of ca) -yes solitary--adrenalectomy multiple--systemic Tx -no--3 3.risk ca -<4cm--repeat CT/MRI 6mo ->6cm--adrenalectomy

Adrenal gland short note by S.Wichien (SNG KKU)

Adrenal sx Lap.adrenalectomy -<6cm 1.lat.transabdo.approach -lat decubitus -10mm *4 Rt adrenal -divide rt triangular -rarely need mobilize hepati colon -retract liver medial -iden rt adrenal v--clip Lt adrenal -fan retract spleen -mobilize splenic colon -reflect spleen,tail pancreas -aware inf phrenic v, join lt adrenal v 2.post.retroperitoneal approach -more direct assess adrenal -avoid abdo adhesion -can bilat sx, not reposition -limit space -unsuit for >5cm -prone jacknife -dissecting balloon

Open/lap.assist adrenalectomy -Adrenocortical ca -malignant phaeochromocytoma 1.Ant approach -exam abdo.cavity -bilat sx -midline/bilat subcostal incision 2.Post approach -avoid adhesion -comorbid pt -unsuit >6cm -prone position -hocky sitick/curvilinear incision -extend to latissimus dorsi -excised 12 rib 3.Lateral approach -best for obesed -large tumor -lateral position 4.Thoracoabdo.approach -en bloc resection (>10 cm)

Nelson synd -30% of bilat adrenalectomy -progress growth of pre exist pitu T -inc ACTH -hyperpigment -visual field loss