tumori hipofizare

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Tumorile hipofizare


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Tumori hipofizare Definitie

Clasificare histopatologica si neuroradiologica Tablou clinic Investigaii

Hormonologie Imagistic

Diagnostic pozitiv si diferential Etiologie si patogenie Anatomie patologica Fiziopatologie

Evolutie, complicatii Tratamentul tumorilor hipofizare

Obiective: etiologic, fiziopatologic, substitutiv Mijloace terapeutice Urmrirea eficienei terapiei

Concluzii


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Tumori hipofizare

Definitie: Adenoame hipofizare benigne, clinic secretante

/nesecretante, intraselare /cu extensie supraselara, rareori ADK

hipofizare, insotite de complicatii endocrine (hipopituitarism,diabet zaharat), neurologice sau oftalmologice.


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Clasificare

Histologic (MO): cromofobe - 70%, PRL 50%

acidofile15%, GH si PRL

bazofile15%, ACTH

Adenoame secretante:20% GH acromegalie

35% PRL prolactinom

7% GH + PRL

7% ACTH + MSH boala Cushing si sd Nelson

20% LH, FSH, TSH

Adenoame nesecretante:

10% cu celule foliculostelate, oncocitom


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Clasificare


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Clasificare neuroradiologica

Hardy:

Stadiul Imicroadenom, < 10 mm

IImacroadenom, cu expasiune

IIIinvaziv localizatIVinvaziv difuz


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Tablou clinic

Sindrom endocrin:Acromegalie/ gigantism;

sd amenoree-galactoree;

Cushing; Melanodermie;

Hipopituitarism

Sindrom neurologic:Sd Neurooftalmic

Leziuni n cranieni III, IV, VI

HIC (cefalee, varsaturi, edem papilar)

Rinoliquoree

Epilepsie temporala

Sindrom metabolic:

DZ secundarobezitate / casexie HPT



Tablou clinic

Sindrom endocrin:Acromegalie/ gigantism;

sd amenoree-galactoree;

Cushing; Melanodermie;

Hipopituitarism



Acromegaly:Outside

Normal blood pressure

Normal breathing patterns

Normal sleep pattern

Increased risk of cardiovascular disease Respiratory conditions and intrinsic

lung disease

Sleep apnea

Without Acromegaly With Acromegaly



Acromegaly: Outside



Sindrom tumoral



Paraclinic - Imagistic

Radiografia de sa turca




Tomografia computerizata



Prolactinom invaziv Craniofarigiom



IRM normal

Lechan RM. Neuroendocrinology of Pituitary Hormone Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987



IRM normal

Modified from Lechan RM. Neuroendocrinology of Pituitary Hormone

Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987




IRM



Tumori hipofizare (Prolactinom)

Amenoree-galactoree

Efect de mas (SChO, obstructie nazal)

Hipopituitarism

PRL: 5700 ng/ml



Craniofaringiom


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Metastaze hipofizare


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Paraclinic - Imunoassay

GH OGTT 0, 30, 60, 90, 120 min (ACM) : 1 ng/ml

PRL 20 ng/ml 100 ng/ml 10000 ng/ml

ACTH, cortizol in DXM ON, 2x2, 2x8

LH si FSH la femeile la menopauza

T4, T3, fT4, fT3 , TSH

Teste bazale si dinamice: exces- supresie;

deficit clinic- inhibitie


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Reacia antigen-anticorp

Ag + Ac* Ag Ac*


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Reacii in sisteme competitive


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Reacii in sisteme necompetitive

Ag voluminos Ac. de captura Ac. de semnal


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Specificitatea anticorpilor i reactivitatea

ncruciat


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IGF-1: variaii cu vrsta i sexul


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Circadian Rhythm in Plasma ACTH and Cortisol in a Clinically Healthy

Adult Women Sampled at 20 minute Intervals

Plasma ACTH

1.1

2.2

3.3

4.4

5.5

6.6

ACTH(mU/L)

*

* *

*

*

*

*

*

*

*

*

**

*

*

* *

12:00 16:00 20:00 00:00 04:00 08:00 12:00

Cortisol(mU/L

)

Plasma Cortisol

138

276

414

552

690

828

12:00 16:00 20:00 00:00 04:00 08:00 12:00

*

*

** * *

*

**

* * *

*

Time (Clock Hour)


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Precauii n evaluarea hipopituitarismului

Obezitate (GH, cortizol n ITT)

Diabet zaharat (valori GH>, IGF1)

Anorexia nervoasa icaexie (GH>; E2/T)

Hipercorticism endo sau exogen (TSH, FSH, LH, TSH


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Diagnostic diferential

Sa turca larga

constitutional, LCR, vase (anevrism de carotida, sunturi

intercavernoase), chist dermoid, arahnoidian, tumori- gliom optic

Galactoreeahiperprolactinemii functionale

Acromegalieacromegaloidie

Melanodermie

Sd Cushing si sd Cushingoide

Insuficiente primare: gonade, mixedem, Addison


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Diagnostic diferential

Sa turca larga Acromegalie


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Model al tumorigenezei hipofizare

Heaney & Melmed, Endocrine related cancer, 7, 2000


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Fiziopatologie

Hormon excesiv/ deficitar

Sindrom tumoralcompresii N II, III, IV, VI

Evolutie: reevaluare in timp 6 luni, 1 an, 3 ani, 5 ani

Complicatii: neurooftalmice, metabolice, HTA,

hipopituitarism, apoplexie hipofizara


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Tratament

Obiective:distrugerea celulelor tumorale, blocareasecretiei autonome, pastrarea functiei

Principii: dg detaliat

Metode: expectativa (incidentalom), chimioterapiespecifica, chirurgia hipofizei, radioterapie


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Tratament etiologic

Chimioterapie specifica Agonisti Dopaminergici: Bromocriptina, Cabergolina Agonisti Somatostatinergici (SMSa) Lanreotide, Octreotide Dopastatine

Analogi GH (Pegvisomant)

Chirurgie transsfenoidala

Radioterapie (! - poate induce hPit) stereotaxica gamma knife


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Tratament substitutiv

Principii:

Ordinea (axa vitala!)

Administram Hh. gl. inta

Rata secreiei, ritm

CSRCortizon acetat (20-30 mg) sau Pdn, alternndcu ACTHa(+ sinteza Androgeni, Cortizol i trofic)

Tiroidian (T4, T3, 100 mg/zi); rhTSH (n KK tir)

Gonadic: (E2/Pg); Fertilitate: (FSHa, LHa) diferit la fa de

GH (opional, costuri)


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tumori hipofizare

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