Copy of Lecture 3, Hypophyseal Tumoral Pathology

8/9/2019 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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Copy of Lecture 3, Hypophyseal Tumoral Pathology