3.Curs Adenoame Hipofizare Si Hipopituitarism Studenti

7/25/2019 3.Curs Adenoame Hipofizare Si Hipopituitarism Studenti

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TUMORILE HIPOFIZARE SITUMORILE HIPOFIZARE SI

HIPOPITUITARISMULHIPOPITUITARISMUL

Dr. Galoiu Simona


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CUPRINSCUPRINS Tumori hipofzare Acromegalia Prolactinomul Boala Cushing Tireotropinoame Gonaotropinomul Incientalomul hipofzar !ipopituitarismul


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Lechan RM., Endocrinology and Metabolism Clinics 16:4755!1, 1"#7

ANATOMIA HIPOFIZEIANATOMIA HIPOFIZEI


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TU"#RI !IP#$I%AR&TU"#RI !IP#$I%AR&'D&$INITI&'D&$INITI&

tumori in ma(oritatea cazurilor )enigne*aenoame+ *,- carcinoame in literatura+

secretante sau nesecretante situate intraselar sau cu e/tensie supra' 0i

paraselar1


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EPIDEMIOLOGIEEPIDEMIOLOGIE

234 in tumorile intracraniene Pre5alenta6 782

Autopsii '9 ' microaenoame Imagistic6 24 in pac. :ara

simptomatologie clinica


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ETIOLOGIEETIOLOGIE Tumori )enigne6 aenoame hipofzare

cranio:aringioame meningioame !iperplazie hipofzara ;lactotro:e *sarcina+

tireotro:e


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C>ASI$ICAR&C>ASI$ICAR&

microaenoame *, 2 cm+macroaenoame *? 2 cm+

Clasifcarea !ar@ ;moifcata

' microaenom sa turcanormala

I ' microaenom intrahipofzarin5azia :ocala a peretelui sellar

II ' macroaenom intrasellar saturca i:uz largita< :ara in5azieIII ' macroaenom in5azie sellara

0i8sau estructie localizataI ' macroaenom in5azie 0i8sau

estructie sellara i:uza


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C>ASI$ICAR&C>ASI$ICAR&

Secretante G! *23 4+ acromegalie 8 gigantism

PR> * 4+ amenoree ' galactoree

ACT! *2 4+ )oala Cushing

TS! *!< 8 G!< PR>< TS! *muteclinic+

*4+ Enull cell aenomaF

monohormonale

plurihormonale ; e/. G!'PR> *mammosomatotro:e+

G!< PR>< TS!<


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PROPORTIA DIFERITELORPROPORTIA DIFERITELOR

TIPURI SECRETORIITIPURI SECRETORII


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Hipofza;hipopituitarism Chiasma optica ;piererea perceptiei culorii

rosii< hemianopsie )itemporala< c5aranopsietemporosuperioara< scotoame< cecitate

Hipotalams' tul). e termoreglare< e apetitINICPARAC>INIC

2. !ipersecretia tumorala G! in #GTT PR> Cortizol upa inhi)itie cu D" :TH< TS! estraiol 8testosteron< $S!< >!

. !ipopituitarism

Teste )azale6 cortizol< :TH< estraiol 8testosteron< $S!< >! Teste in inamica ;stimulare ;G! ; ITT< Arg

'cortizol ' ITT


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I"AGISTIC ' R"N


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I"AGISTIC ' CT

CG 24 years

Macro PRMSSE

PRL=3,100 ng/ml


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N$AN$A

BBorsors IIonon, M, 37, M, 37 anan,, SC!"SC!", #$% I& SSE, #$% I& SSE


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L! ' ICCL! ' ICC $S! ICC $S! ICC

ANAT#"I& PAT#>#GICA 'I"UN#!IST#C!I"I&


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DIAGN#STIC P#%ITIDIAGN#STIC P#%ITI

Clinica Imagistic

!ormonii hipofzari nesupresi)ili*teste e inhi)iJie+ !ormonii hipofzari efcitari *teste

e stimulare+ ComplicaJii6 *o:talmologiceDIAGN#STIC DI$&R&NTIA>

2. R/' sinromul e sa turca goala' mal:ormatii arterio5enoase *ane5rism carotiian+IA 'D&$INITI&

Boal1 eterminat1 e hipersecreJiae G!< ce uce la cre0tereaaccentuat1 a scheletului 0i 5iscerelor.

eterminata in peste 34 in cazurie un aenom hipofzar< rareori fino secreJie paraneoplazica e G!R!

*tumori pancreatice+.


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ACR#"&GA>IA ;DAT& &PID&"I#>#GIC&ACR#"&GA>IA ;DAT& &PID&"I#>#GIC&

Inc(en)a* 3'4 ca+r/ 1 000 000 /an

Pre-alen)a* . / 1 000 000

Raa moral)* 2'3 ola)a general 11.

Cauza de deces

n=56 (66+12 ani)

Frecvena % (n) nacromegalie

Frecvena % npopulaia general

%oala coronariana &'.& (15) &5.6

%oli cerebro*asc+lare 14.' (#) 1!

lte boli cardio*asc+lare 16.1 (") 7.&

-eolasme &1.4 (1&) &1.'/+mori hio0iare 5.4 (')

ccidente #." (5) #.4

ltele 1!.7 (6) &7.5

Oauppinen'"a=elin et al.Q Clin &nocrinol "eta)< Qul@ 3< *L+6H


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C>INICC>INIC

2.Semne eterminate e e/cesul e G!6

m1rirea minilor 0i picioarelor moifcarea fzionomiei tegumente groase< mate< umee< cu miros

particular 5isceromegalie6 cariomegalie< cu agra5area

prognosticului

respirator6 moifc1ri ale 5ocii< o)strucJie nalt1 etract respirator 0i apnee e somn cario5ascular6 cariomegalie< !TA secunara macrogenitosomie.


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.Semne comune cu alte tumorihipofzare< eterminate e compresie*sinrom neurologic+6

sinrom neuroo:talmic ce:alee

semne clinice e insufcienJ1hipofzara

C>INICC>INIC


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C>INIC


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PARAC>INIC

Pro)e uzuale G! minim in #GTT ? 2 ng8ml IG$'2 crescut pentru 5arsta si se/ PR> Cortizol< :TH< estraiol8testosteron!

&/. o:talmologic6 C< A< $#

ACROMEGALIA CRITERII DEACROMEGALIA CRITERII DE


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ACROMEGALIA 'CRITERII DEACROMEGALIA 'CRITERII DE

DIAGNOSTIC SI EFICIENTA ADIAGNOSTIC SI EFICIENTA A

TRATAMENTULUITRATAMENTULUIIncienta6 382< pre5alenta6 -82.


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I"AGISTIC


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DIAGN#STIC DI$&R&NTIA>DIAGN#STIC DI$&R&NTIA>

2. cre0terea nontumoral1 a G! seric6insufcienJ1 hepatic1 8 renal1< malnutriJia#GI&$I%I#PAT#>#GI&

&:ecte epenentee IG$'I

&:ecte inepenente

e IG$'I


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TRATA"&NTTRATA"&NT

Chirurgical ;e prima intentie "eicamentos

$ Analogi e somatostatin ;octreoti ;sc< 3

ug8zi 'sanostatin >AR

'lanreoti PR< autogel$ Agonisti opaminergici ;)romocriptinaUTI&< C#"P>ICATII>UTI&< C#"P>ICATII

Cario5asculare ;cariomiopatia< !TAsecunara< aritmii< 5al5ulopatiiPR#>ACTIN#"U>

Tumora hipofzara secretanta e PR> cea mai :rec5enta tumora hipofzara Clinic6 oligomenoree ; amenoree

galactoreein:ertilitateTDSin:ertilitate

semne ate e tumora ;SC!# ?2 ng8ml? ng8ml

Teste e insufcienta hipofzara &/. o:talmologic Imagistic

DIAGN#STIC DI$&R&NTIA> A>T&DIAGN#STIC DI$&R&NTIA> A>T&


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DIAGN#STIC DI$&R&NTIA> ; A>T&DIAGN#STIC DI$&R&NTIA> ; A>T&CAU%& D& !IP&RPR#>ACTIN&"I&CAU%& D& !IP&RPR#>ACTIN&"I&

2. fziologice ' sarcina< alaptatul' stimularea peretelui toracic< stress< somn

. meicamentoase6

i. )locanJi ai receptorilor opaminergici6 haloperiol< :enotiazine*clorpromazin1+< omperione< metocloprami antiepresi5e

triciclice.ii. rezerpin1< metilopa *:ac epleJie central1 e opamin1+

iii. estrogenii

i5.TR!< cimetiin1< 5erapamil< opioie.

. leziuni hipotalamice sau ale ti(ei hipofzare

'tumori< infltrate' secJiunea sau compresia ti(ei< traumatism< chirurgical

H. alte tumori hipofzare< cu secreJie mi/t1< e PR> 0i G! 8 ACT!

3. hipotiroiism primar *secreJie reacti5a e TR!+-. insufcienJa renal1 cronic1< insufcienJ1 hepatic1


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O C O / 0PROLACTINOMUL /PRM0


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PROLACTINOMUL /PRM0PROLACTINOMUL /PRM0

-DIAGNOSTIC-DIAGNOSTIC

PR> ? 3 ng8mlmacroprolactinom

PR> ? 3ng8ml PR" PR> ? ng8ml PR" 8

meicamente 8sarcina

PR" , ng8ml PR"8altecauze

ETIOLOGIAETIOLOGIA


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ETIOLOGIAETIOLOGIA

HIPERPROLACTINEMIEIHIPERPROLACTINEMIEI

"acroprolactinemia6 Big')ig )ig ?"acroprolactinemia6 Big')ig )ig ?34 $re34 $reuenc@6 24 *2-87H+

PRL

adenoma

stalk section

syndrome

iatrogenic

idiopathic

macroprolact

inemia

PCOS

2-

7

2L

7

-

7


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$I%I#PAT#>#GI&


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TRATA"&NTTRATA"&NT

"eicamentosV

$ Agonisti opaminergici ;)romocriptina 3' mg8zi

' ca)ergolina


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PR>

Diam. tumoral

,2cm

DA

responsi5

neresponsi5

5inecatW raioterapi

e

chirurgie

? 2 cm< cu SC!#? 2 cm< :araSC!#

ani

temozolom

i

HIPERPROLACTINEMIEIHIPERPROLACTINEMIEI

Ghi(l E!(oc$i!# Soci#t12Ghi(l E!(oc$i!# Soci#t12

,3**,3**

C#C

PR" maligne


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B#A>A CUS!INGB#A>A CUS!ING

D#f!i4i#6 hipersecreJie relati5autonoma e ACT! e c1tre unaenom hipofzar.

De regul1 un microaenom< caresecret1 ACT! 0i uneori peptieeri5ate in P#"C< eterminn

hiperplazie corticosuprarenal1)ilateral1 cu hipersecreJie e cortizol.


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C>INICC>INIC

&erger ros'-olacee

Pele s:re, ;raglae calara

Sca(erea ;ore msclar ro


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PARAC>INICPARAC>INIC

Cortizol pl. ora 7< ora H< C>U< 2L #!CScrescuti

D" 2mg o5ernight

D" mg / D" 7 mg / ACT! crescut

Teste uzuale Test la CR! Cateterizare sinus pietros in:erior

Suspiciune clinic1 *'3 semne


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pclinice+

Cortizol li)er urinar

D" 2 mg p.o. o5ernight*ora +

!ipercortizolismreacti5

Sinrom pseuo'CushingX

Sinrom Cushing

D" / mg p.o.

cortizol pl. ora H


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GHIDUL ENDOCRINE SOCIET5/,3360 DE DIAGNOSTIC AL/,3360 DE DIAGNOSTIC AL

SINDROMULUI CUSHINGSINDROMULUI CUSHING

74

Boala

Cushing


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. Bertagna et al. 8 Best Practice K Research Clinical &nocrinolog@ K"eta)olism *+

TRATA"&NTTRATA"&NT


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TRATA"&NTTRATA"&NT

!ipofzectomie selecti5a Suprarenalectomie )ilaterala

Complicatii6 sinrom Nelson Iraiere hipofzara supra5oltata "eicatie a(u5anta ;=etoconazolICATIIC#"P>ICATII


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C#"P>ICATIIC#"P>ICATII

Sinromul Nelson Insufcienta corticosuprarenala recii5a


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$eelers< Q Clin &nocrinol "eta)ICATIIC#"P>ICATII


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C#"P>ICATIIC#"P>ICATII

!ipogonaism ; pe termen scurt

'pe termen lung ' osteoporoza

In:ertilitate

Date e tumora

Date e tratament

G#NAD#TR#PIN#"U>


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G#NAD#TR#PIN#"U>

Aenom hipofzar secretant egonaotropi ;$S!< >!<

3040% in tumorile hipofzare

SecreJia nepulsatil1 e gonaotropitumorali )locheaz1 e regul1 :uncJiagonaic1.

Uneori se poate mani:esta cuhiperstimulare o5ariana8testiculara Tratament ;chirurgical< raioterapie

TIRTR#PIN#"U>TIRTR#PIN#"U>


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TIRTR#PIN#"U>TIRTR#PIN#"U>

Tumora secretanta e TS!< este ceamai rar1 tumor1 hipofzar1 *,4+.

Cli!ic6$ gu0a i:uz1$ tireoto/icoz1$ sinrom tumoral hipofzar.

La%o$ato$$TS! normal sau crescut$ :TH< :T crescute

TRATA"&NT


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TRATA"&NT

chirurgical 'hipofzectomie "eical' #ctreoti sau >anreoti raioterapie hipofzar1

Tiroiectomia sau antitiroiienele e

sintez1 agra5eaz1 e5oluJiasinromului tumoral


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HIPOFIZAREHIPOFIZARE


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HIPOFIZAREHIPOFIZARE

Ghiul &nocrine Societ@< 22

!IP#PITUITARIS"U>!IP#PITUITARIS"U>


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!IP#PITUITARIS"U>!IP#PITUITARIS"U>

1. e0icit+l de : nanism la coil a0ectare metabolic8 la ad+lt

&. e0icit+l de gonadotroi: hiogonadism hiogonadotro

la coil: +bertate int9riat8

la 0emeie: ano*+laie cronic8, amenoree, in0ertilitate la b8rbat: /3, in0ertilitate

'. e0icit de /3: hiotiroidism central

4. e0icit de C/: ins+0iciena adrenal8 sec+ndara

5. e0icit+l de ;RL: agalactia ostart+m



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S.C. &rrige et al. 8 Raiotherap@ an#ncolo

NY 73

AC ;nYL7< RRY2.H3 "< . $H tumori maligne intracraniene

CAUZE DE DECES LACAUZE DE DECES LA


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CAUZE DE DECES LACAUZE DE DECES LAPACIENTII CUPACIENTII CU

HIPOPITUITARISMHIPOPITUITARISM

N8*,69 pac+ chipopitita$ism

SUPRA.IETUIREA PACIENTILORSUPRA.IETUIREA PACIENTILOR


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%ueger< Q Clin &nocrinol "eta)< #cto)er

2< L*2+

SUPRA.IETUIREA PACIENTILORSUPRA.IETUIREA PACIENTILORCU INSUFICIENTA CORTICOTROPACU INSUFICIENTA CORTICOTROPA

SU7STITUITASU7STITUITA

&tiologie


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&tiologie

1 GE#E>IC?*

@ Ins; o; mllA* ma)HEX1* (sla+a seo'oca(e;c (e G!Pit-1, Prop1* (e;c (e G!, PRL,>S!, gona(oro

@ Ins; o; +olaA*

ma)* KAL* s(r %LLM%## = ogona(sm ogona(oro anosmeDAX1*ogona(sm ogona(oroola+e a(renalA congenalAGH1* nansm o;+ar ;amlal

AVP-NEUROFIZINAII* (a:e ns( cenralTSH-* oro(sm cenral

LH- * ogona(sm ogona(oroFSH- * ogona(sm ogona(oroGHRH* nansm o;+ar ;amlalGnRH* ogona(sm ogona(oroTSH reeptor* oro(sm cenral

&tiologie


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&tiologie2 >DM"R%L?*

@ >(sem:rola+ce* crano;arngom, csr arano(e sa

csr (er- (n nga Rae@ > are* ;nc)onale sa ne;nc)onale@ > e measace* (e orgne renalA, mamarA

3 I#$IL>R%>I&?@ Bol granlomaoase* sarco(o+a, granloml eo+no;l, >BC, s;ls,

granlomao+a Fegener@ !socoDRCIC G"%L 6emy sella8

&tiologie I6


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&tiologie

3. ASCU>ARZ6

MApople/ia pituitar[ *Sr. Sheehan+M"al:ormaJii 5asculare6 ane5risme

-. AUT#I"UNZ6Mhipofzita lim:ocitar[Mefcitul izolat e ACT!

L. IRADI&R&

7. TRAU"ATICZ6MsecJiune e ti([ pituitar[6 sdr. de hipofz izolat

*insu:. aenohipofzar[ ia)et insipi central hiperprolacti

. IATR#G&NZ6Mchirurgia hipofzeiMraioterapie hipofzar[

I6in5asi5eS, n;erlae

3 e;c (e >S!* oro(sm cenral

4 e;c (e %C>!* ns;cen)a a(renalA

5 e;cl (e PRL* agalaca osarm

I#SD$ICIE#K% %E#"!IP"$I%RI#SD$ICIE#K% %E#"!IP"$I%R


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KKGL"B%LGL"B%L


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Defcitul e G!


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Defcitul e G!


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Nanismul hipofzar


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Nanismul hipofzarMgreutate 0i talie normale la na0tereMsc[erea 5elocit[Jii e cre0tere postnatal[

Mefcitul izolat e G!6nanism armonic'D#PAMCloniina 3'2 ani

Mtratament6 G!r 3'3\g8=g8zi s.c.

G!, 2ng8ml


88/100

Cauze e nanism


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@ Malnr)a s (er-area emo)onalA@ Sara mcA ;amlalA@ nJr+erea cons)onalA a creNer s :erA)@ e;cl (e G! 69O8@ Rear(l (e creNere nraern 67,5O8@ sla+ scelece 6acon(rola+a, ocon(rola+a8

@ S(r genece (smor;ce 6>rner, on8@ Bol cronce*IRC, :oala celacA, mal;orma) car(ace,@ Bol en(ocrne* oro(sm

oararo(smS(r Csng

@ Rasml caren)al comn sa - re+sen@ S(r (e re+sen)A la G!

nansm Laron* G! cresc,IG$'I scA+

H4

Defcitul e G! al aultului


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MSc[erea energiei 5itale 0i a st[rii e )ineMIzolare social[MDispozitie epresi5[MAn/ietate crescut[

MCre0terea aipozitaJii centrale 0i sc[erea masei musculareMSc[erea sensi)ilit[Jii la insulin[ cu alterarea toleranJei la glucoz[MCre0terea >D> colesterolului si a apoB sc[erea !D>

MSc[erea ensitaJii minerale osoase cu risc crescut e :ractura pa

MSc[erea masei miocaiceMCre0terea f)rinogenului plasmatic 0i a inhi)itorului tisular e plasMAteroscleroza accelerat[



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]nainte e tratament

Dup[ tratament


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INSU$ICI&N^A AD&N#!IP#$I%AR_


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INSU$ICI&N^A AD&N#!IP#$I%AR_DG. PARAC>INIC

1 >ese :a+ale* mAsrarea o;+ar N a glan(elor )nA

@ %C>!, Cor+ol am@ >esoseron am sa esra(ol $S!, L!@ PRL@ G!, IG$'I@ >S!, ;>4, ;>3

2 >ese (namce *

@ >es (e smlare la nslnA 6I>>8enr arecerea re+er-e(e G!6nQ7ng/ml8 s cor+ol 6nQ21g/(l8

@ >es (e smlare c glcagon, can( I>> ese conran(ca@ >es (e smlare c %C>! enr arecerea re+er-e

a(renale 6(g (; c :%((son8


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98/100

TRATA"&NTU> INSU$ICI&NT&I


99/100

!IP#$I%AR& Tratarea cauzei Insufcienta CSR6 !irocortizon 23' mg8zi in '

prize sau prenison 3'2 mg8zi Insufcienta tiroiiana6 le5oth@ro/ina< 2! pentruresta)ilirea :ertilitatii

Defcitul e G!



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Defcitul e G! al aultuluiCriterii e selectie pt. tratamen

MDefcit se5er e G!6 ITT6 G!,ng8ml dublate prezenta uneipatologii hipofzare semnifcati5e

MAlte efcite hormonale e(a su)stituite

MClinica6Sc[erea se5er[ a calit[Jii 5ieJiiSc[erea ensit[Jii minerale osoase *T,'2DS+Sc[erea toleranJei la e:ortProfl cario5ascular cu risc crescut

MDoza meie

3.Curs Adenoame Hipofizare Si Hipopituitarism Studenti

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