Tumorile hipofizare

Corin Badiu, 2010

Tumori hipofizare
Definitie Clasificare histopatologica si neuroradiologica Tablou clinic Investiga ii
Hormonologie Imagistic

Diagnostic pozitiv si diferential Etiologie si patogenie Anatomie patologica Fiziopatologie Evolutie, complicatii Tratamentul tumorilor hipofizare
Obiective: etiologic, fiziopatologic, substitutiv Mijloace terapeutice Urm rirea eficien ei terapiei

Concluzii
Corin Badiu, 2010

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.

Corin Badiu, 2010

Clasificare
Histologic (MO): cromofobe - 70%, PRL 50% acidofile 15%, GH si PRL bazofile 15%, ACTH Adenoame secretante: 20% GH p acromegalie 35% PRL p prolactinom 7% GH + PRL 7% ACTH + MSH p boala Cushing si sd Nelson 20% LH, FSH, TSH Adenoame nesecretante: 10% cu celule foliculostelate, oncocitom

Corin Badiu, 2010

Clasificare

Corin Badiu, 2010

Clasificare neuroradiologica
Hardy: Stadiul I microadenom, < 10 mm II macroadenom, cu expasiune III invaziv localizat IV invaziv difuz

Corin Badiu, 2010

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 secundar obezitate / casexie HPT

Corin Badiu, 2010

Tablou clinic
Sindrom endocrin: Acromegalie/ gigantism; sd amenoree-galactoree; amenoreeCushing; Melanodermie; Hipopituitarism

Corin Badiu, 2010

Gigant si pitic

Cel mai scund om din lumechinezul He Pingping, (73 cm) si cel mai inalt turcul Sultan Kosen (246 cm).

Corin Badiu, 2010

Acromegaly: Outside

Without Acromegaly
Normal blood pressure Normal breathing patterns Normal sleep pattern

With Acromegaly
Increased risk of cardiovascular disease Respiratory conditions and intrinsic lung disease Sleep apnea

Corin Badiu, 2010

Acromegaly: Outside

Corin Badiu, 2010

Tablou clinic
Sindrom endocrin: Acromegalie/ gigantism; sd amenoree-galactoree; amenoreeCushing; Melanodermie; Hipopituitarism

Corin Badiu, 2010

Corin Badiu, 2010

Sindrom tumoral

Corin Badiu, 2010

Paraclinic - Imagistic
Radiografia de sa turca

Corin Badiu, 2010

Paraclinic - Imagistic
Radiografia de sa turca

Corin Badiu, 2010

Paraclinic - Imagistic
Tomografia computerizata

Corin Badiu, 2010

Prolactinom invaziv

Craniofarigiom

Corin Badiu, 2010

IRM normal

Lechan RM. Neuroendocrinology of Pituitary Hormone Regulation. Endocrinology and Metabolism Clinics 16:475-501, 1987 Corin Badiu, 2010

IRM normal

Modified from Lechan RM. Neuroendocrinology of Pituitary Hormone Regulation. Endocrinology and Metabolism ClinicsBadiu, 2010 Corin 16:475-501, 1987

Paraclinic - Imagistic
IRM

Corin Badiu, 2010

Tumori hipofizare (Prolactinom)

Amenoree-galactoree Efect de mas (SChO, obstructie nazal ) Hipopituitarism PRL: 5700 ng/ml

Corin Badiu, 2010

Craniofaringiom

Corin Badiu, 2010

Metastaze hipofizare

Corin Badiu, 2010

Paraclinic - Imunoassay
Teste bazale si dinamice: GH PRL exces- supresie; deficit clinic- inhibitie OGTT 0, 30, 60, 90, 120 min (ACM) : 1 ng/ml 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

Corin Badiu, 2010

Corin Badiu, 2010

Reac ia antigen-anticorp antigen-

Ag + Ac* ' Ag Ac*

Corin Badiu, 2010

Reac ii in sisteme competitive

Corin Badiu, 2010

Reac ii in sisteme necompetitive

Ag voluminos

Ac. de captura

Ac. de semnal
Corin Badiu, 2010

Specificitatea anticorpilor i reactivitatea ncruci at

Corin Badiu, 2010

Corin Badiu, 2010

Oral Glucose Tolerance Test

Acromegaly: positive & differential diagnosis Diabetes Mellitus

Oral glucose 75g GH peak level > 1 Qg/L

Corin Badiu, 2010

IGF-1: varia ii cu vrsta i sexul

Corin Badiu, 2010

Circadian Rhythm in Plasma ACTH and Cortisol in a Clinically Healthy Adult Women Sampled at 20 minute Intervals
.

Plasma ACTH
6.6

Plasma Cortisol
828

* * * *** * * *

* * * *

* *

ACTH (mU/L)

5.5 4.4 3.3 2.2 1.1

Cortisol (mU/L)

690 552 414 276 138

* * * * * * * * *
00:00 04:00 08:00

* *

12:00

16:00

20:00

00:00

04:00

08:00

12:00

12:00

16:00

20:00

12:00

Time (Clock Hour)

Corin Badiu, 2010

Corin Badiu, 2010

Corin Badiu, 2010

Precau ii n evaluarea hipopituitarismului

Obezitate (GH, cortizol n ITT) Diabet zaharat (valori GH>, IGF1<) Insuf. Renala cronica (GH, PRL, TSH, FSH, LH, CLU>) Anorexia nervoasa i ca exie (GH>; E2/T<, cort>) Hipercorticism endo sau exogen (TSH, FSH, LH<) Depresie (cortizol>, TSH<)

Corin Badiu, 2010

Diagnostic diferential
Sa turca larga
constitutional, LCR, vase (anevrism de carotida, sunturi intercavernoase), chist dermoid, arahnoidian, tumori- gliom optic

Galactoreea hiperprolactinemii functionale Acromegalie acromegaloidie Melanodermie Sd Cushing si sd Cushingoide Insuficiente primare: gonade, mixedem, Addison

Corin Badiu, 2010

Diagnostic diferential
Sa turca larga Acromegalie

Corin Badiu, 2010

Model al tumorigenezei hipofizare

Heaney & Melmed, Endocrine related cancer, 7, 2000 2010 Corin Badiu,

Corin Badiu, 2010

Anatomie patologica
NFA, TSH & FSH IR

BI, M, 37 y, IV SSE NFA, Sindrom Optochiasmatic

TSH(c/s) = 3.54/2,18; LH (c/s) = 6.96/1.53 mU/ml, FSH (c/s) = 39.66/18 3.54/2,18;

TSH - ICC

FSH ICC
Corin Badiu, 2010

Fiziopatologie
Hormon excesiv/ deficitar Sindrom tumoral compresii N II, III, IV, VI Evolutie: reevaluare in timp 6 luni, 1 an, 3 ani, 5 ani Complicatii: neurooftalmice, metabolice, HTA, hipopituitarism, apoplexie hipofizara

Corin Badiu, 2010

Sindromul Nelson
Boala Cushing post SRectomie bilaterala defrenaj hipofizar, melanodermie, macroadenom invaziv.

Corin Badiu, 2010

Tratament
Obiective: distrugerea celulelor tumorale, blocarea
secretiei autonome, pastrarea functiei Principii: dg detaliat Metode: expectativa (incidentalom), chimioterapie specifica, chirurgia hipofizei, radioterapie

Corin Badiu, 2010

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
Corin Badiu, 2010

Corin Badiu, 2010

Corin Badiu, 2010

Tratament substitutiv
Principii:
Ordinea (axa vitala!) Administram Hh. gl. inta Rata secre iei, ritm

CSR Cortizon acetat (20-30 mg) sau Pdn, alternnd cu ACTHa (+ sinteza Androgeni, Cortizol i trofic) Tiroidian (T4, T3, 100 Qg/zi); rhTSH (n KK tir) Gonadic: (E2/Pg); Fertilitate: (FSHa, LHa) diferit la fa de GH (op ional, costuri )
Corin Badiu, 2010

CONCLUZII
Evaluare imagistica de inalta rezolutie Evaluarea bazala, dinamica in inhibi ie sau stimulare Integrarea rezultatelor clinice, biochimice, imagistice. Tratament specific, selectiv

Corin Badiu, 2010