copy of lecture 3, hypophyseal tumoral pathology
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LECTURE 3
HYPOPHYSEAL
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HYPOPHYSEAL
Frequency: 10-15% of intracranial tumors.
Necroptic studies 6-23% asymptomatic hypophyseal tumors.
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neurological symptoms tumoral
syndrome!
functional symptoms endocrinesyndrome!
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direct compression
intracranial hypertension
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secondary to the alteration of
hypophysial
and peripheral "lands function.
Functional consequences may #e: secretory de ciency secretory excess
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1. +, ,+ / + ) ,+2. ), 4 , , +4 43. ), +/ + 77 4 84 ), 4
9. 8 +)4 N 7 8 4++4+5. ;, 7 )4 N6. 7 +, +4 4. +8/4N 7 + N,+
/ypophyseal fossa
Hypophyseal fossaHypophyseal fossa
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the roof:
the "land is surrounded #y ? dura mater@(
formed #y a re$ection of the dura attached to theclinoid processes A the diaphra"ma sellae.
)he optic chiasm lies : 5-10 mm a#o*e the diaphra"ma sellae and anterior to the stal .
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9 sta"es /ardy!:1- microadenoma B10 mm diameter!C
sella turcica dimensions are not chan"edC2- macroadenoma D10 mm diameter!C
'ith E'ithout suprasellar e tensionC sellar dimensions could #e modiGedC
3- macroadenoma- 'ith local invasion C sella turcica - bigger than normalC 'ithE'ithout suprasellar e tension
9 -macroadenoma- di use invasion 'ithE'ithout suprasellar e tension.
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denoame hipoGHare in*aHi*e I ima"ini radio"raGce
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Adenoame hipofizare invazive imagini radiografice Adenoame hipofizare invazive imagini radiografice
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acidophilic adenomas
#asophilic adenomas
chromopho#ic adenomas
)here is not a direct corelation #et'een:- the histologic appearence and
- a speciGc endocrine acti*ity.
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denomas:
Functional
Non-functional
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/ypophyseal tumors:
most are adenomas
adenocarcinoma
a rare condition
*ery in*asi*e.
it may produce: J/(8 ( )/.
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hypophyseal tumors
occasionally associated to adenoma- in other endocrine "lands
8ancreas( 8arathyroid
A deGnin" Multiple Endoc ine !eoplasia syndrome (MEN).
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"#$ of hypophyseal adenomas
a./ypothalamic cause: hypothalamic-dopamine-inhi#itor tonus-
decrease
#./ypophyseal ori"in: "ene mutations
spontaneous or induced follo'ed #y hormone or "ro'th cellular
factors in*ol*ement
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#asophilic icroadeno a ==->0 %!macroadenoma *ery rare!- hi"h local in*asi*epotential.
8atho"enesis I un no'n- 2 theories:a. /ypothalamic theory - it could not ha*e #een pro*ed
#. /ypophyseal theory :
)/ secretion capacity - increased" corticotropic cells sensi#ility to hypercortisolism Idecreased
sustained #y: inhi#ition of this hyperfunction 'ith de amethasone
synthetic "lucocorticoid!.
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*ery a"ressi*e acroadeno a
occurs in patients presentin": #ilateral adrenalectomy for ushin"Ks
disease
recei*in" lo' or inadequate doses of"lucocorticoid therapy.
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slo'ly e*oluti*e
may ha*e perisellar de*elopment - 'ithneurolo"ical symptoms
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25 % of hypophyseal macroadenomas inmen:
lon"-e*oluti*e primary hypo"onadism
promotor effect on "onadotropic cellshyperplasia :a. "onadotropin- releasing hormone
Jn /! - stimulates especiallyalpha -chains synthesis
#. activine - stimulates beta -chainssynthesis
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less than 1 %ay #e:
a. pri ary thyrotropinoma- secretes )+/that may produce hyperthyroidis I
a e condition
#. secondary thyrotropinoma: a reacti*e hyperlasia of thyrotropic cells ccurs as a result of pri ary hypothyroidis
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7o not present systemic secretorycapacity.
/istolo"ical studies electronomicroscopyand immunohistochemical studies!
adenomatous cells secrete "lycoproteic
su#units #eta-F+/ and alpha-F+/C /! I'hitout systemic eMect.
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#isual $eld modiGcations:
occur in lar"e tumors 'ith sup asellae tension
due to optochiasmatic system: compression( inGltration or intracranial hypertension.
90 % of cases- bite poral he ianopsy
L *ery precocious si"n - pale optic papilla .
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%ranial nerves dysfunction ( ( and !-
due to late al e tension:
loss of pupilar reacti*ity
#lepharoptosis
diplopia
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phenoidal sinusitis due to:
in'asion of sphenoidal sinus C
the tumor may "ro' into the sinus
'ithout symptoms.
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Hypophyseal apople*y:acute he%o ha(ic infa ct represents a neurosur"ical emer"ency.+ymptoms: intense headache( *omitin"( *isual alterations and e*en #lindness( ophthalmople"iaC /emorrha"ic +F
+ometimes - a real ?autohypophysectomy@
appears
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8artial
)otal
7eGciency depends on:
tumoral "ro'th speed
patientKs age
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)here is a strict order in deGcit installation:"onadotrops /(F+/!
"ro'th hormone J/!
thyrotropin )+/!
corticotropin )/!
8rolactin 8 ! is rarely decreased.
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8articularity LLL A a sec etin( adenomaJ/(8 ( )/-secretin" adenoma ! may#e accompanied #y symptoms speciGc for
de$ciency of other hor ones.4 emple:
crome"aly and hypogonadis
+ enorrhea and "alactorrheaushin"Ks disease( "alactorhea andhypogonadis .
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+ecretin" adenomas produce speciGcdisease:
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de*elops on lactotropic hypophyseal cells
predominant in 'omen- possi#ly due tohyperestro"enism
t could #e:
Microprolactinoma more frecquent in
!o en !
Macro prolactinoma more frequent inen !
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linical manifestation depend on:
"ender
a"e
duration of e*olution.
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Functional eMects I usually preceed
tu%o al %ass e)ects
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/yperprolactinemia inhi#its pulsatilerelease of gonadoliberine (resultin" in:
alteration of pu#ertal se ualiHation process
alteration of menstrual cycle in 'omen
alteration of se ual dynamic in men
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4Mects on a ary gland :"alactorrhea in 'omen(rarely in men
"ynecomastia in men.
)he association a%eno hea*(alacto hea A hyperprolactinemia in
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8rolactin directly stimulates adrenal corte*
andro"en e cess -
/yperandro"enic syndrome in 'omen: +e#orrhea
cne
/ypertricosis
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Neurologic sy pto s - supraselar e tension :
intracranial hypertension
optochiasmatic compression syndrome'ith E 'ithout ophtalmople"ia .
more frequent in: men and 'omen at menopause.
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'aboratory $ndings :
&asal 8 D 200 n"Ed Amacroprolactinoma 100-200n"Ed in the a#sence of pre"nancy! A
microprolactinoma oradenoma O compressin" the hypophyseal stal
B 100 n"Ed I dru"s( polycystic o*ary( hepatic E renalchronic failure
dyna%ic tests I useful 'ith limited dia"nostic*alue!: inhi#itin" tests - le*o-dopa(#romocriptine(nomiphensine
stimulatin" tests - ) /(chlorpromaHine(domperidone.
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7ru" induced : estro"ens( neuroleptics( cimetidine( *erapamil( enalapril( metoclopramid
diopathic
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J/ e cess
+omatoli#erine e cess J/- /! -rarely
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1. hypophyseal secretin" adenoma:
- J/- J/ and 8- plurihormonal
2. ectopic hypophyseal tumors: - in the sphenoidal sinus
- parapharyn"ial
3. e trahypophyseal tumors:- pancreatic -pulmonary- o*arian- mammary
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1. hypothalamic tumors
2. ectopic tumors:
lun" cancer(
adrenal adenoma
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lmost all #ody structures are aMected.
4fects of J/: directly or throu"h its eMectors- somatomedins or insuline-
li e "ro'th factors- JF- ! :in adults- promotes "rotesque and e a"erated"ro'thA acrome"aly
in child and adolescent- accelerates "ro'thrateA "i"antism
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J/ e cess:cannot #e reduced only at estheticaspects
J/ is in*ol*ed in: intermediary meta#olism
*isceral functionality
cellular replication process
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increase in basal etabolis ( hyperhydrosis( heatintolerance
glucose intolerance and e*en diabetes ellitushypercalce ia (hypercalciuria(nephrolithiasishyperphosphore ia (increased al alinephosphatasealteration in hypophyseal hor ones secretion :
hyperprolactinemia( hypopituitarism(
dia#etes insipidusthyroid alterations: nodular "oiter( hypothyroidismpossi#le association to other endocrine neoplasia=multiple endocrine neoplasia syndrome 4N!
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P carpal tunnel syndrome
P hypertrophic neuropathy
P ner*e compression
P pro imal myopathy
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o' sonorous( nasonant *oice
o#structions of upper respiratory tract
increased lun" *olume
apnea durin" sleep
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thic #one corte
de"enerati*e arthropathy
rachidian spondylosis
#one me"aliHation
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GIGANTO ACROMEGALY
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GIGANTO-ACROMEGALY
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thic s in( folds
s'eatin"( se#orrhea( pi"mentation(hirsutism
papilomas( lipomas
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Cha acte isticsCha acte istics
+lo' e*olution delayed dia"nosis 10
years!
7ia"nosis is made 'hen somatic modiGcationsare irre*ersi#le --- in most cases LLL
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increased serum J/:
permanent or intermittent more often!
increased JF-1: constantly
inhi#ition test 'ith "lucose: in normal persons: J/ - inhi#itedC in acrome"aly - a parado al increase of J/ *alues
) / and J/- / tests - similar response.
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Sotos+ synd o%e
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Pa(et+s disease
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Pachyde %ope iostosis
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My,oede%a
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Simple prognati m !con tit"tional#
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ACROMEGALOID FACE
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$ar%e -&e'er yn(rome !)emicorporeal )ypertrop)y#
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>0 % of cases I icroadeno a
responsi#le for ushin"Ks disease
symptoms and si"ns - due to: e cessi*e )/ adrenal corte
hyperstimulation increase in"lucocorticoid le*el
rare disease 5 casesE1millionEyear!
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Clinical featu es
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-Moon*face./
telan(iectasia
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omplications:
cardio*ascular(
infectious(
dia#etes mellitus
IMAGING STUDIES IN
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on*entional s ull -ray: lateral or anterior )omo"raphy
n"io"raphy
8neumoencephalo"raphy) scanC 'ith iodinated contrastsu#stance - more precise!
'ith "adolinium - more precise!atheterism of petrous inferior sinuses
IMAGING STUDIES IN*Y$O$*YSEAL ADENOMAS
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E% ll
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E%pty sella
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TREATMENT
NE"ROS"RGERYRA!IOT ERAPY
ME!ICAL T ERAPY
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T anssphenoidal -microadenomas
T ansf ontal -suprasellarde*elopin"adenomas
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T anssphenoidal for microadenomas!:
microscopic *ie'
economic resection
minimal postoperati*e complications and
ne"lecti#le mortality 0(1%!
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T ansf ontal - suprasellar de*elopin" adenom LLLMo tality *0#*01$
8ostoperati*e complications :
hypophyseal insuQciency(
liquorrhea(
#lindness(
ocular ner*es paralysis.
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8ostoperatory e pectancies:
normaliHation of in*ol*ed hormonele*el
normaliHation of other hormones.
* ti l di th
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con*entional radiotherapy R-rays and
"amma-rays
radioacti*e isotopes implantation Sttrium >0 or ridium 1>2
irradiation 'ith hea*y particles
multifascicular irradiation 'ith o#alt 60-Jammanife
)he last t'o methods are the most used.
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adiotherapy - useful in:
J/-secretin" adenomas #efore and aftersur"ery!
PRL Isecretin" adenoma I esistent toradiotherapy.
)/-secretin" adenomas #efore and aftersur"ery!
LLL ny inopera#le adenoma
G %% 2 if
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Ga%%a2nife
ste eotacticadiosu (e y
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7ama"e of: optic chiasma( optic ner*es(
cranial ner*esischemia leadin" to cere#ral necrosiscere#ral edema
pulmonary acute edemacon*ulsionshypophyseal insuQciencyradiation dermatitis
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o%oc iptine dopamine a"onist! :
decrease in 8 synthesis
inhi#ition of cellular multiplication
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+ide eMects: nausea( *omitin"(
hypotension( anaphylactic shoc .
)reatment: starts 'ith lo' doses- 1(25m"Ed( pro"ressi*ely increase to 10-20 m"Ed orally
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ther dru"s: 8er"olide isurid ;uin"olide 4 J N,
8eriodical assessment of: serum 8 le*el( sellar dimensions( campimetry
% i i 20 30 "Ed
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o%oc iptine - 20-30 m"Eda#er"olinum 1- 9"E 'eeSo%atostatine 4Oct eotide/ anreotide 5 :
orally( intranasal(
s.c.( i.m.- once e*ery 2-9 'ee s. decrease in:
tumoral siHe in 50 % of cases serum J/ le*el in =0 % of cases
o%oc iptine6 So%atostatine
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