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ACTHGH

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Pituitary tumors

• Pituitary tumors are the most common

diseases of the pituitary gland

• Benign and monoclonal - arise from single

type of anterior pituitary cells

• Variable presentation

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Function

Hyersecretion

Insu!!iciency

Size

Microadenoma

Macroadenoma

Pituitary tumors

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Pituitary Adenomas

Commonnest causes

Ma/ority are 0yersecretin"

Endocrinolo"ic a1normality

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Pituitary Hyersecretion

• PRL most commonly

secreted by adenoma

hyperprolactinemia

• H !cromegaly

• !"#H "ushing$s disease

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ACROMEGA2Y

• H-secreting pituitary adenoma %nd fre&uency

• "linical manifestations '

"hronic H hypersecretion o(ergro)thof bone is the classic feature particularly ofthe s*ull and mandible

• !dults ' linear gro)th does not occur+because of prior fusion of the epiphyses oflong bones

• "hildhood , adoloscence igantism

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ACROMEGA2Y• tiology' e.cessi(e pituitary H secretion

• Se. incidence e&ual

• /ean age at diagnosis is appro.imately

01 years

• 2uration of symptoms usually 3-41 years

before the diagnosis established

• 5ncreased morbidity and mortality if

untreated slo)ly progressi(e and

spontaneous remission

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Clinical mani!estations o!

acrome"aly• /anifestation of H .cess

• 2isturbance of other endocrinefunction

• Local manifestation

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acrome"aly/anifestation of H .cess

!cral enlargement+ soft tissueo(ergro)th+ hyperhydrosis+ lethargy

or fatigue+ )eight gain+ paresthesis+

6oint pain+ hypertrichosis+ goiter+hypertension

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!dults ' linear gro)th does not occur+ because of prior fusion of the

epiphyses of long bones7 "hildhood , adoloscence igantism

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acrome"aly 2isturbance of other endocrine function'

hyperinsulinemia+

glucose intolerance+

irregular or absent menses+ decreased libido+

hypothyroidism+

galactorrhea+

gynaecomastia+

hyperadrenalism

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acrome"alyLocal manifestations

nlarged sella

Head ache

Visual deficit

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2a1oratory !indin"

H hypersecretion 8 41 ng 9mL

postprandial hyperglycemia+serum insulin is increased+

ele(ated serum phosphorus+

hypercalciuria

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5nitial steps diagnosis ' :euro-opthalmologic

e(aluationand

:euro-radiologic studies )ith/R5

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Ima"in" study• Plain films'

Sellar enlargement ;<1= cases>+

nlargement of the frontal+ ma.illary

sinuses and the 6a)

#hic*ening of the cal(arium

5ncreased thic*ness of the heel pad• /R5

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5ncrease in hell pad thic*ness

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3isual Field De!ects

• Bitemporal hemianopsia

• Visual loss

• Large tumor diplopia+

cranial ner(e dysfunction

;:555+5V+V5>

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ffects of pituitary

tumors on the(isual apparatyus

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Dia"nostics

• 2a1oratory !indin"s4 H increase 41 ng9mL ;:? 4-3 ng9mL>+ ppplasma glucose+ serum insulin+ serumphosphor + hypercalciuria

• Ima"in" studies4 Plain film <1= casessellarenlargement

nlargement of 6a)+ ma.illary sinuses+increased soft tissue bul*

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Treatment• Remo(al or destruction of pituitary tumor

• Re(ersal of H hypersecretion

• /aintenance of normal pituitary function

• "riteria for ade&uate respons H@ 3 ng9mL

Remission@ % ng9mL

• 5nitial therapy transphenoidal micro surgery7

• Radiation th9 reser(ed for patients )9 inade&uatresponses to surgery , medical therapy

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Treatment• Surgical treatment ' for small or moderate Asize

tumors ;@ %cm> transphenoidale Surgery isthe treatment of choice for microadenomas;<1= cure>

• /edical treatment'Somatostatin analog

ctreotide acetate ;Sandostatin> , Lanreotide

;Somat uline>

the therapy of choice )ithresidual H hypersecr7 Collo)ing surgery

• Radiotherapy

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HIPOTHALAMUS

PITUITARY

SOMATOSTATINGHRH

DOPAMINE-AGONIST

DRUGS:BROMOCRIP

TINE,CABERGOLINE

SOMATOSTATINAGONIST :

OCTREOTIDE

IGF-1

GH-RECEPTOR

GH-RECEPTOR

ANTAGONIST:

PEGVISOMANT

GH

IGF-1 :Insulin like !"#$% &'($"!

GH

ACTH . ti Pit it

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ACTH .ecretin" Pituitary

Adenoma

'Cus0in" Disease+

• Har(ey "ushing 4<D%

• !"#H Hypersecretion

bilateraladrenal hyperplasia

• Spontaneous hypercorticolism;"ushingsyndrome>7

• 22' !drenocorticosteroid e.cess-ctopic !"#H syndrome and adrenaltumors

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Clinical Features

• nset insidious+ usual %1-01 y+ C'/ 'E'4+

ctopic !"#H /'C'D'4

• "entral besity+ hypertension+ glucose

intolerance+ gonadal disfunction+ moon

facies+ osteopenia+ muscle )ea*ness+

(iolaceous striae+ hirsutism+ acne+poor

)ound healing+ fungal infection+

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Dia"nosis 5 Treatment

• Basal plasma !"#H

• #reatment '

Surgical treatment' microsurgery Radiotherapy' "on(entional radiotherapy

/edical therapy' no drugs supresses pituitary

!"#H secretion7 F Getoconazole to inhibit adrenal steroid biosynthesis+

F /etyrapone+ aminoglutethimide reduce cortisol

hypersecretion

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EMPTY .E22A .YNDROME

• tiologyCongenital Subarachnoid space

e.tends into the sellaturcica )ith

cerebrospinal fluid enlargement+

remodelling ;Congenital ,after pituitary

surgery or radiation therapy, postpartum

pituitary infarctin (sheehan syndrome)

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Clinical !eatures and Dia"nosis

• /idlle aged+ obese )omen

• Hypertension+ rhinorrhea+ (isual field

impairment

• 2iagnosis' /R5

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CRANIOPHARYNGIOMA

• "hildren and adolescence

• 8E1= hypothalamic-pituitary deficiencies

H deficiency most common+ gro)th

retardation+ gonadotropin deficiency

• Symptom' intracranii pressure+ decreased

(isual acuity

• 2iagnose'/R5

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Pituitay Insu!!iciency

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Pituitay Insu!!iciency

• Panhypopituitarism

classic manifestation

of pituitary adenomas

Hypogonadism c9

nRH screened

CSH9LH to e.clude

primary gonadal failure

• #SH or !"#Hdeficiency is relati(ely

unusual

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Posterior Pituitary

Antidiuretic 0ormon 'ADH6 3asoressinAntidiuretic 0ormon 'ADH6 3asoressin ) )

Dia1etes Insiidus ' 2eficient !2Haction

.ynd Inaroriate ADH ' High plasma

!2H concentration

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DIA7ETE. IN.IPIDU.

• 5s disorder of )ater balance caused by

nonsmotic renal losses of )ater

• tiology ' deficient argenine (asopressin

;!VP?!2H> secretion ;central> or end organunresponsi(enes to !VP ;nephrogenic>

• !VP is released from cells in the posterior

pituitary gland increase )ater permeability at

the distal tubule and collecting duct of the

nephron

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DIA7ETE. IN.IPIDU.

• Classi!ication

"entral 25' Hypophysectomy+ idiopathic+

familial+ tumor9cyst+ granuloma+autoimmune

:ephrogenic 25' chronic renal disease+hypo*alemia+ hypercalcemia+ familial+ etc

22' Primary polydipsy ;Psychogenic+compulsi(e )ater drin*ing>

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DIA7ETE. IN.IPIDU.

.ymtoms4#hirst+ polyurea+ daily urine (olume 8D L

Hypernatremia )ea*ness+ altered mental status+

coma+ seizures Signs' Physical e.amination isusually normal

2a1oratory E%aluation4

Spesific gra(ity @47141Urine osmolality @D11 m sm9*g

Hypernatremia

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Dia"nosis

• High Plasma osmolality

• Urine osmolality reduced

• Iater 2epri(ation Spesific-ra(ity @47113;%11mosm9Gg of )ater>

• Plasma Vasopressinlo) in :eurogenic

25 and :9high in nephrogenic 25+ lo) inpsychogenic polydipsia

• !2H Radioimmunoassays

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Treatment

% goals' replace the )ater deficit , treatunderlying abnormality

• "entral 25

2esmopressin acetat ? !VP analog 22!VP>• :ephrogenic 25

Underlying disorder should be treated if

possible+ diuretic+ prostaglandin synthesis

inhibitors+ amiloride

. d ! i i t

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.yndrome o! inaroriate

secretion o! ADH '.IADH+

tiology/alignant lung disease+ #B"+ lymphoma+ ":S trauma+ drugs;clofibrat+ chlorpropamide+ H"#>+ H5V infection+ ndocrinediseases ; adrenal insuff+ my.edema>

"linical /anifestationHyponatremia' 5 'diarrhea+ nausea+ (omiting+ neuropsychiatricsigns+ muscular )ea*ness+ headache+ lethargy+cerebral edema

#reatmentCluid restriction

2iuretics

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Disorders o! adrenocortical !unction

Adrenocortical 0yer!unction

lucocorticoids

!ldosteronism /ineralocorticoids

Virilizing tumors !ndrogens

Ceminizing tumors strogens

Adrenocortical 0yo!unction

Hypopituitarism lucocorticoids

Hypoaldosteronism /ineralocorticoids

Hypopituitarism !ndrogens

strogens

Cus%in8s s)n*!"+e

A**is"n8s *ise'se

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ADRENA2 INCIDENTA2OMA.

• /asses found incidentally during

radiographic imaging of the abdomen

• 5ncidence ' 17D3-07DJ= in general

population

5n (aluation such mass'

• 5s the mass benign or malignantK

• 2oes the mass secrete hormones or mass

disfunction

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DD ADRENA2 INCIDENTA2OMA

• 7eni"n4

:on hormone secreting ;lipoma+cyst+ganglioneuroma+ adenoma>

Hormone secreting ;pheochromocytoma+aldosteronism+ subclinical "ushing$s syndrome

• Mali"nant

!drenocortical carcinoma

/etastatic neoplasm

2ymphoma

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ADRENA2 INCIDENTA2OMA.

• .i#e is imortant4

!drenal masses 80cm more li*elymalignant surgical resection should

be consideration• #he great ma6ority ; E<=> are 1eni"n$

non !unctionin" masses

• A !ull 1ioc0emical 9or:u should becompleted before surgery is done

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CU.HING8. .YNDROME

• "hronic glucocorticoid e.cess Symptoms ,Physical features "S

Iatrogenic CS ; "hronic glucocorticoid

therapy>' most commonly Spontaneous CS '

Pituitary ;"ushing disease>

!drenal !"#H secretion non pituitary tumor ;ectopic !"#H Syndrome>

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CU.HING8. .YNDROME

Classi!ication !"#H-dependent

Pituitary adenoma ;"ushing disease> M1=:onpituitary neoplasma ;ectopic !"#H>

!"#H-independent5atrogenic ;glucocorticoid+ megestrol acetat>

!drenal neoplasma ;adenoma+ carcinoma>+Hyperplasia

Cactitious

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CU.HING8. .YNDROME"ushing$s syndrome suspected

(ernight 4 mg 2S#

!/ cortisol 8 47E ug9dl !/ cortisol @47E ug9dl :ormal

%0 hours urine free cortisol

:ormal le(ated

Repeat if high ndocrinology

inde. of suspicion consultation

CU.HING8. .YNDROME

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"ushing$s syndrome established

ACTH IRMA

@3 pg9mL 841 ug9mL

"# adrenals /R5 pituitary

Unilateral mass Bilateral enlargement 5PSS :ormal !bnormal

"RH test

Pea* !"#H Pea* !"#H 5PS'P@47E 5PS'P8%71

@41pg9ml 8%1 ug9dL

Adrenal sur"ery Ectoic ACTH Pituitary .ur"ery

CU.HING8. .YNDROME

P.EUDO CU.HING8

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P.EUDO CU.HING8s

.YNDROME

• besity

• "hronic alcoholism

• 2epresion

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Post adrenalectomyHirsutism

Disorders o! adrenocortical

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Disorders o! adrenocortical

insu!!iciency

• 2eficient adrenalproduction of

glucocorticoid and mineralocorticoid

!drenocortical insufficiency

N Primary adrenocortical insufficiency

;!ddison$s disease>

N Secondary ' deficient pituitary !"#H

secretion+ glucocorticoid therapy

;most common>

Addi 8 Di

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Addison8s Disease

• tiologi' tbc ;prior 4<%1>+ !utoimmuneadrenalitis adrenal atrophy ;E1=>

!ssociated other immunologic and

autoimmune endocrine disorders+ !52S+malignant disease

• Rare+ female 88+ D1-31 year

•"linical features' )ea*ness+ fatigue+anore.ia+ )eight loss+ hyperpegmentasi+hypotension+

Addison8s disease

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Addison8s disease• 2a1oratorium 4

Hiponatrimia- hiper*alemia ;classic>

Radiologis 9"# Scan

• Dia"nosis

Basal adreno*ortical steroid:ormal

Rapid !"#H stimulation test

!"#H plasma

• Treatment4

Replacement therapycortisol

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ACTH

Aldosterone

Renin substrate

Renin

Angiotensin I

Angiotensin II K

EBV

/a6or factors regulating aldosterone secretion BC;ffecti(e Blood Volume>

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Primary Hyeraldosteronism

• !ccounts for about 17M= of cases ofhypertension+ Iomen 88+ unilateral

adrenocortical adenoma ;"onn$s

syndrome+ MD=>+ %M= bilateral• Hyperaldosteronism' hypertension+

hypo*alemia+ al*alosis

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Primary Hyeraldosteronism

"linical finding' Hypertension+ muscular

)ea*ness+ paresthesias+ headache+

polydipsia+ polyuria+ moderate

hypertension ;malignant is rare>Laboratory finding' Serum potassium lo)+ %0

hours urine collection aldosterone

5maging' "#-scan

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Primary Hyeraldosteronsm

#reatment' Laparoscopic

adrenalectomy+ Spironolactone+

antihypertensi(e agent

"omplication' Renal damage

Prognosis' 5mpro(ed by early

diagnosis and treatment+ only %=malignant

Diseases of adrenal medulla

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Diseases of adrenal medulla

PheochromocytomaPheochromocytomas are rare (<0,2% ofhypertensive), cathecolamine-producingtumor of neurochroman cells.

!traadrenal Ph sympathetic gangliaare called Paraganggliomas

"ncidence #-$th decades,autosomaldominat hereditary, malignant 0-&%cases 'ypertension is caused ye!cessive plasma level epinephrine ytumor located either or oth adrenals

any*here along sympathetic nervus

P0 0 t

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P0eoc0romocytoma

• Symptoms and Signs Usually lethal unless diagnosed and treated

se(ere headache+ perspiration+ palpitation+an.iety+ tremor+ tachycardia

!ttac* cyanosis+ facial pallor

"lassical symptomatic triad' headache+s)eating+ palpitations

• Laboratory findingUrinary cathecolamines+ metanephrine+creatinine+ Urinary V/!

P0 0 t

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2ocalisation• "# scanning

A (erall accuracy <1=-<3= for adrenal tumours A Less accurate for e.tra adrenal tumours

• 5sotope scintigraphy ;/5B scanning>

A 4D45-/5B stored in chromaffin granule A Sensiti(ity <<= A Calse negati(e 44= A Calse positi(e %=

7lood and Urine analysis A Plasma catecholamine le(els 8 4111micrograms A Urinary V/! and /etanephrine le(els

P0eoc0romocytoma

P0 0 t

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• Surgery Preoperati(e preparation

• #o control hypertension , pre(ent "VS

complications7

• !lpha adrenergic bloc*ade

A Pheno.ybenzamine 41 mg &ds 4-% )ee*s before

surgery

A Beta bloc*ade propanolol 41 mg &ds %-D days• 5ntraoperati(ely

• Phentolamine

• Sodium nitroprusside

• #reatment

P0eoc0romocytoma

P0 0 t

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• #reatment Laparoscopic remo(al of thetumor treatment of choice+ open

laparatomy

• Prognosis

2epends early diagnosis is made

P0eoc0romocytoma

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