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
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Tablou clinic
Sindrom endocrin:Acromegalie/ gigantism;
sd amenoree-galactoree;
Cushing; Melanodermie;
Hipopituitarism
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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
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Acromegaly: Outside
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Sindrom tumoral
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Paraclinic - Imagistic
Radiografia de sa turca
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Paraclinic - Imagistic
Tomografia computerizata
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Prolactinom invaziv Craniofarigiom
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IRM normal
Lechan RM. Neuroendocrinology of Pituitary Hormone Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987
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IRM normal
Modified from Lechan RM. Neuroendocrinology of Pituitary Hormone
Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987
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Paraclinic - Imagistic
IRM
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Tumori hipofizare (Prolactinom)
Amenoree-galactoree
Efect de mas (SChO, obstructie nazal)
Hipopituitarism
PRL: 5700 ng/ml
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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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