SISTIM ENDOKRIN

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SISTIM ENDOKRIN Dr. Hj. RESMI KARTINI, MS

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Transcript of SISTIM ENDOKRIN

  • SISTIM ENDOKRINDr. Hj. RESMI KARTINI, MS

  • Homeostasis seluler diatur :Sistim saraf Sistim endokrin terutama hipotalamus ( pituitari )Dan sel neuroendokrin APUD

    HormonKelenjar endokrin target organinteraksi melalui reseptor

  • Reseptor - reseptorSurface membrane receptorsHormon polipeptide ( pituitary )KatekolaminCytoplasmic reseptorSteroidNukleusIntranuclear reseptorThyroid hormonSitoplasma

  • HIPOTALAMUS

    KONTROL

    KELENJAR ENDOKRIN PERIFERFEED BACK MECHANISMPITUITARY HORMON STIMULATORY INHIBITORY

  • Pituitary gland

    ANT:adenohipofisis Rathkes PouchPost : Neuro Hipofisis Floor Ventr IIIPost - Vasopresin ( ADH ) -OxytocinAnt -Asidofil somatrroph GH mammotroph PrL -Basofil Corticotroph Proopiomesamocortin acth,msh, endorfin,lipotropin Tirotrof TSH Glikoprotein Gonadotrop: FSH,LH -Khromofob

  • PATOLOGIPrimer : Lobus Ant PostAdenohipofisis Hiper / Hipopituit 1.Tropic hormon / Hipopituitary : 75 % destruksi -tumor non fungsional -Infeksi -Hipotal (jarang)

    2.Local effect - rare - Sela tursika besar --- Xray,CT,MRI Expansile lesion ( neoplasma )

  • - Defek Expanding dis.pit Chiasma opt / N. II Bitemporal hemianopsia visual impairment- Sangat jarang Tek intrakranial( Headache, nausea, vomitus Neoplasma )

  • HiperpituitariAdenomaKarsinoma (Hipotalamus Stimulasi >> Pituiti)----- jarangAdenoma MonoclonalSingle hormon Manifestasi klinikPlurihormonalPrimitif / Pluri potent cells

  • Morfologi

    Mikro adenoma ( < 10 mm )Mikro adenoma ( > 10 mm )Mikro adenoma : 40 % unselected autopsi. single ,multipel multiple foci hiperplMakro adenoma : Ekpansi dpt erosi tulangSella enkapsul poorly diliputi retikulin chiasma optikum syaraf kranialDasar otak,sinus cavernosus, tlg sfenoid

  • Mikroskopis :

    Uniform sel poligonal tersusun sheet,cord,nests.Stroma vascularisasi bertambahNecrosis ischemik,psammoma bodiesHemoragik pituit apoplexyMitosis /pleomorfik ? Malignancy metastasis

  • PITUITARY ADENOMA

  • PITUITARY ADENOMA

  • Somatotropic ADENOMASSomatotro. acidophilic macro adenoma GH Akromegali Gigantisme jarangHiperplasiaAkromegali Adult Megali :akral,Kepala, tgn, Kaki,jaw,lidah Soft tissue

  • Gigantisme : ChildrenSel matur uniform --- granulatedSebagian pleomorfik asidofil1/3 BSM Prl bimorfusPrl granul pada sel neoplastik tidak terdapat pada darah

  • ProlactinomasSparsely granulatedHiperplasia jarangHiperprolactinemi lesi hipotal drug impair dopaminergic trans mission (metildopa,reserpin) estrogen terapi Hipogonad laki 2 --- impoten,infertil peremp --- galactorrhea amenorrhea

  • 2/3 macro adenoma granulated acidophilic cells1/3 Micro adenomaKalsifikasiSel uniform sela tursika

  • Corticotroph tumors

    basophilik mikroadenoma ACTH ------ Hipersekresi kortisol adrenalHiperplasia / multiple micro adenoma cushings diseasePro opio melanocortincrookes hyaline changeskhromofobic less well defined cortisol excessSilent tumor besar induce local changes

  • Other functioning AdenomasGonadotroph tumor 6%Laki 2 FSH LH hipogonadPerempuan Gonadotropin sekresi - wl pun LH dapat dideteksi serum BesarMikros : Laki-laki Pleom, wanita :uniform small cell

  • CARCINOMA

    DiferensiasiDiagnosis Matastase : KGB Bone Hati dll

  • Disorders associated with HypopituitarismGerm cell TKraniofaringiomaGliomaKetiga diatas lesions of hypothal,ant hipofis hipopituitary 75 % destruksi klinikTumor klinik : Diab-insipidus Growth Acceleration Delayed puberty

  • 90% hipopituitproses destruksi adeno hipophisisTU: Nonsecretory adenomas Sheehans pit.necrosis Empty sella tursikaNeoplasma .metas.Pituit apopleksi chemorDisruption blood supply by sistemic arteritis / or trSinus venosus cavernosusDistruksi inflamasi lob ant by sarcoidosis / inf,surgical / radiation ablation metab.dis

  • NON SECRETORY CHROMOPHOBE PITUIT ADENOMA

    25 -30 %Klinik efek lokal - Kelainan lapangan pandangan - Sakit kepala - hipotensi --- one of target endoc organ UNDER pituit control ( HIPOTIROID HIPOGONADISM )

  • MORFOLOGIBesar silent bertahun-tahunNull cell adenomaOncocytoma sparsely granules lessionImunohistokimia FSH, / hormon, LH

  • SHEEHANS SYNDROME / NECROSIS PITUIT POST PARTUMHamil pituitary besar Hemorr vasospasmenekrosis.iskemik/ infark lobus anterior Shock

  • DICSickle cell anemiaTr. Sinus cavernosus arteritis temporal, traumatic injury pembuluh darah, DM (lama)

  • 95-99 % destruksi lobus anterior defisiensi gonadotropic laktasi menurun (puerperium)Defisiensi TSH atau hipotiroidACTH (insufisiensi adrenocortical)Infark : soft, pale fibrosis

  • EMPTY SELLA SYNDROMEHernia arachnoid deffect diafragma sellaTekanan CSF atropi pituitary empty sella

  • SINDROMA PITUITARY ANTERIORJarangTerutama lesi hipotalamus suprasellerDisfungsi ADH defisiensi diabets insipidusArginin vasopresin:oksitosin kontraksi uterus partus stim glandula lactating

    Neoplastik : tumor supraseller, metas Ca, abses, meningitis, TBC, sarcoidosis (inflamasi hipotalamus-hipofisial)Surgical radiasi hipotalamus-hipofisialSevere head injuryIdiopatik

  • Hypothalamik suprasellar tumors Neoplasma jrg GLIOMA / Craniopharyngioma

    Hipofungsipituitary anteriorHiperfungsiDiabetes insipidusCraniopharyngiomaDari sisa rathkes pouchPada sella, suprasellar Anak, dewasa mudabenign

  • MORFOLOGI3-4 cm, solid encapsule, kistik multilokuler, kalsifikasiMikroskopis :

  • ADENO HIPOFISIAL HIPOFUNGSILorain levi syndromeGH / hormon lain HipoglikemiagonadotropinKraniofaringiomaFrohlichs syndromeObesitasPerkembangan sex arrestMental sub normal,Growthtumor

  • Prepubertas--- growth pituitary dwarfism sexual retard

  • HIPOGONADISMAmenoreLoss of axilla and pubicSterillityOvarium atropi, genital atropiTestis atropi, sterillityLoss of axilla, pubicHipotiroid TSHHipoadrenal ACTHPanhipopituit Simmonds

  • TSHIodine in diet iodine+tirosin T3 sekresi abs stimulasi monoiodo oleh TSH oleh TSH andbowel diiodotirosinT4 blood

  • TIROIDTirotoksikosis (hipertiroidism)HipotiroidismDifus / fokal enlargement (Goiter) tidak ada hubungan antara lesi morfol dan klinik

  • TIROTOKSIKOSISHipertiroidism, Free T3 , T4 Hiperfungsi tiroid (Graves disease) hipertiroidismNon hiperaktif kelenjar tirotoksikosisSindrom :NervousnessBB (nafsu makan baik)PalpitasiHeat intoleranceRapid pulseWarm skinFatigabilityEmosi labilKelemahan ototPerubahan haidBMRTremor tanganPerubahan mataKelenjar tiroid membesar

  • MORFOLOGIVasodilatasi : Periferheat lossSirkulasi hiperdinamik general kulit : warm, moist, flushedhipertiroidperubahan mataGraves disease---proptosis perubahan imunologiInflamasi sekunder jaringan retroorbital jantung TakhikardiPalpitasiKardiomegaliAritmia, fibrilasi supraventrikuler

  • GRAVES DISEASE

  • THYROTOXIC CARDIOMIOPATHYFokus infiltrasi limfosit, eosinofil, interstisial, fibrosis ringan, fatty changesManifestasi lain: Otot skelet : atropi, fatty infiltrasi, infiltrasi limfosit fokal Hati : minimal fatty changes, fibrosis periportal, infiltrasi limfositOsteoporosis general lymphoid hyperplasia dg lymphadenopathy

  • Graves Disease< 40 th (85%)HipertiroidDiffuse toxic hyperplasiaHiperfungsi tiroid Pemberian iodine >> pada kelainan (T3 / T4)tiroid Jod basedows disease

  • HIPERFUNGSI TIROIDT3 T4 sirkulasi tirotoksikosisTerdiri dari :Graves diseaseToxic adenomaToxic noduler goiterKlinik :ExophtalmusProminent tiroidBMR Skin warm, sweaty : heat intoleranceWeakness,hiperkinesia, emotional instabilityBBGlucose tolerance, glukosuriaNadi , Cardial arrhythmia and failure ( orang tua )TSH

  • TIROIDITISTiroiditis interstitialPalpation tiroidSuperior / inferior tiroidjarang direct traumaticImunologi Staf, strep, salmonella, enterobacter, M tbc, jamur (candida, asper, mucor)

  • MorfologiKelenjar besarHasimotoSub acute granulomatous tiroiditisSub acute limfositic tiroiditisRiedel struma

  • HASHIMOTOS THYROIDITIS/ STRUMA LYMPHOMATOSA/ LYMPHADENOID GOITER : Goitrous hipotirNon endemic goter (children)Autoimun diseaseBerlangsung lama hipotiroid hipertiroidhashitoxicosisHashimoto Grave

  • ETIOLOGIAutoimunDefek fungsi tiroid spesifikSupressor T cellAntibodies tiroid peroksidase (antimicrosomal antibodies)AB Spesifik untuk tiroglobulinTSH reseptor

  • MorfologiKelenjar besar, diffuse, asimetris, kapsul intak, coklat noduler, Rubbery tissueMikroskopis :Perjalanan penyakit : usia pertengahanHipotiroidismAtropiStadium dini metabolisme normal, fungsi tiroid, TSH, T3 T4 Prognosa baik: 1-200 lymphomaGoitrous hipotirNon endemic goter (children)Autoimun disease lymphoma

  • HASHIMOTOS THYROIDITIS

  • HASHIMOTOS THYROIDITIS

  • Riedels fibrous thyroiditis /Riedel strumaCausa unknownAtropi/hipertropi, fibrosis,firm,abu 2 caKadang : Retroperitoneal fibrosis( multi fokal fibro sclerosis )Mikros : fibrosis , infiltrasi limposit scant,fol tiroid distorsi

  • Sub acuta granulomatous(de QuervainS )Thyroiditisde Quervains Thy Self limited, inf granule / Giant cell / Gran.thy dekade 2-5 : --- 3: 1Unknown virus onset didahului infeksi virus (mumps,measles,influ,adeno vi,coksackie vi,echo vi) Klinik : demam,tir >>> nyeriMORFOLOGI :Tir > > > /

  • MORFOLOGI

    Tir > > / Fokal asimetrisOpt gland tkena irreg : lokalisasi firm,kuning putih ruberryHistologi :Patchy LamaProses inflam,micro absesLanjut folikel rusak / agregat sel sukar ? sel dari folikel makropagmultinuclear giant cell (koloid) fibrosis gran thyroiditis

  • PERJALANAN PENYAKIT1. Acute systemic febrile, LED2. Nyeri, GL > sore throat , earache3. Nyeri
  • SUB ACUTE GRANULOMATOUS THYROIDITIS (DE QUERVAINS THYROIDITIS)

  • SUBACUTE LYMPHOCYTIC (PAINLESS) THYROIDITIS? GOITROUS HYPERTHYROIDFokus infiltrasi limfositJaringan fibrous interstisialKel tir >> dan Hipertiroid Amerika utara 15 % graveGoitrous >> tdk terdeteksi self limiting disease hipotiroid? T3 dan T4

  • GRAVES DISEASEHipertiroid hiperfungsi difuse hiperplasia goiterInfiltratif ophtalmopathy, dermopathyOpthalmopathy :Lid lagUpper lid retractionEdem periorbitalStareOtot mata lemahproptosisDermopathy:Edem pinggung kaki, tungkaiPlaque like / nodulerLokal myxedema(-) Tirotoksikosis diffuse toxic goiterUSA 12-20 %

  • ETIOLOGI DAN PATOGENESISAUTOIMUNIg G antibodi TSH reseptorThyroid stimulating AB (TSAb)Thyroid stimulating Ig (TSI) HashimotoPada pdrt Graves dpt an.pernisiosa, rheumatoid arthritisMORFOLOGI :Diffuse hyperplasia80-90 grKapsul intactDiameter parenkim lunak daging

  • HISTOLOGICell>> vask Pseudopapil mushroom, folikelKoloid , pucatStroma jaringan limfoid >>, folikel >>general lymphoid hypertrophyPERJALANAN PENYAKIT Muda tirotoksikosis tir > simetrisUptake JOD131 Proptosis >> injury cornea, ulserasi, mata (-)

  • DIFFUSE AND MULTINODULAR GOITERKerusakan output hipertiroid kompensasi kad TSH Hipertropi, hiperplasia epitel folikel Diffuse/ multi nodulerTir > hor < eutiroid1. diffuse non toxic (simple goiter)2. multinod goiter

  • Diffuse non toxic (simple) goiterPembesaran diffuseHiper/hipofungsiENDEMIC GOITERSimple goiter, lokalisasi geografik 10 % populasi pegununganIntake yod hor tir TSH hipertir, hiperplasiaCa, flour goiterMakanan dan sayur

  • NON ENDEMIC / SPORADIC SIMPLE GOITER 8 : 1Pubertas / Dws mudaTSH Kehamilan estrogen TBG 1. defek transport jod T3 T42. defek organifikasi feed back inhibition TSH3.defek dehalogenaseDefek iodotirosin coupling

  • MORFOLOGIStadium hiperplastik, colloid involutionHiperplastikKel >> sedang (100-150 gr)Difuse, simetris, hiperemik Histologi : epitel fol kolumner, generasi fol ,
  • Multi noduler goitersimple goiter multi nod goitersporadik endemiknon toxictirotoxicosis (toxic multi nod goiter) plummers diseaseJarang hipotiroidTiroid membesar DD/neoplastikOlder ok. komplikasi Sel tiroid normal-heterogen perbedaan potensiProliferatifFolikel membesar ruptur hemoragik jar. Parut kalsifikasi

  • Morfologi1. nod hiperplasi folikel, folikel dg koloid banyak2. irreguler scarring3. Hemoragik fokal, deposit hemosiderin4. kalsifikasi fokal pd jar. Parut5. mikrokista

  • Perjalanan PenyakitKlinik penting:Ukuran, lokalisasi massaFungsi abnormal biasa : tirotoksikosisPerbedaan dg neoplasmaCVS pd org tua: fibrilasi atrium, takikardi, heart failure kosmetik, disfagi, stridor inspirasi, penekanan v,kava sup : distensi vena leher dan ekstremitas atas, edem palpebra / konjungtiva, batuk dan sinkope

  • Hemoragik tiroid >>, nyeri dan serakHiperfungsi toksik noduler goiterUptake jod 131 , T3 T4 Akumulasi radioaktif tersebar ke dalam tiroidJarang menyebabkan noduler hiperfungsiTiroid >> 2000gr 1 atau lebih lobus menekan trakea, Oesofagus, dan dibelakang sternum intrathoracic plunging goiter

  • MorfologiMultinod heterogenNod batad tidak tegasJar. Parut tampak sebagian encapsul Penamaan yg salah adenomatous goiter dan multiple coloid adenomatous goiterDD/: neoplasma sukarCT scanUSGMRIBiopsi aspirasiResiko Ca namun data (-)

  • NODULER GOITER

  • NODULER GOITER

  • HipotiroidismHipometabolismeHipotiroid1. selama fase perkembangan, invasi, kretinisme physical, mental retard2. older children/adult myxedemaAkumulasi hidrofilik mukopolisakaridPada jar penyokong edem dan kulit tebal

  • KausaUSA hipotiroid, idiopatik primertiroiditis autoimun atropik15-60% Sustansi ------------TSH reseptor bloking auto ABdapat menghilang spontanTerapiPost tiroidektomi >>

  • CretinismJarangRetardasi fisik dan intelektualPada saat lahir tergantung dr hormonal efeknya terlihat setelah bbrp minggu-bulan2Klinik : kulit kering,kasar Widely set eyes peri orbital bengkak hidung lebar,flattened lidah besarJod -pada perkembangan fetal Agenesis tiroid Defek biosintese kongenital Pertumbuhan skelet terganggu, perkembangan otak Endemik cretinism endemik goiterSporadic cretinism cretinism sporadik atropi

  • MYXEDEMAOlder child diantara kretin dan dewasa dg hipotiroidAdult pelan2 Aktifitas fisik dan mental lambatBicara, intelek Edem preorbitaKulit tebal, kasar, kering, lidah >>Facial feature thikenedLetargik keringat banyak, konstipasi, fungsi motorik => Sebagai kretinMikros : miofiber edem, striae hilang

  • TUMORSNODUSA, adult, 2-4%, nod daerah endemik goiter,90%adenoma, karsinoma 25-35kasus/milionSolitary mass nodules, multinucleated goiter, kista, pembesaran asimetrisNod soliter neoplastikwarm/hot nodul benignaPenderita> non neoplastik

  • ADENOMADari epitel folikel ad. FolikulerMikros : fetal,embrional,simple, colloid, mikro & makrofolMorfologi :DD/ sukar dg multinod goiterBerkapsul, mendesak sekitarHomogen, 3-10 cmHitam abu2, soft fleshyHemoragis, fibrosis sentralNekrosis sentral -- kista

  • Mikroskopis :TrabeculerFetal stroma miksomstousColloidSpindel / atipikalhurthlePerjalanan penyakit :Sloly increase insizeUkuran tertentuCepat besar, nyeri, hemoragisJarang T3 dan T4 tinggicold nodule dapat hot

  • ADENOMA FOLLIKULER

  • ADENOMA FOLLIKULER

  • Malignant tumors USA 1991 1000 , 2/3 perempuan, >1% penyebab Papil Ca 75-85%Fol Ca 10-20%Med Ca 5%Anaplastik Ca jarangPatogenesis :1. Radiasi head and neckPembesaran tonsil,timus,acne, irradiasi pd dekade 2 carcinogenik4-9%infant rad Ca 20 th, 70% bom di jepang2. hashimoto limfomaNon toxic goiter

  • Papillary CaKhas :Hipokhromatik empty nucleidevoid of nucleus (orphann annie eyes)Inti grooveInklusi intranukl eosinofilikpsammomaEncapsulated variantFollikulerTall cell

  • PAPPILARY CARCINOMA

  • Follicular CarcinomaPerjalanan peny :Foll Ca tumbuh lambat lanjut irreg,multinodD/ ditegakkan metas (+)/(-)Prognosis :Ukuran primer?Invasi kapsul +/_Anaplasia?Larger invesive Ca5 th surv rate 30%10 th surv rate 20 %

  • Anaplastic Ca5 %Older, daerah endemik goiterHistologi:Spindle cell Ca dapat fol, papil CaGiant cellSmall cell jarangD/ massa cepat >>Lokal invasive (trakhea)

  • Medullary CarcinomaNeuroendokrin neoplasma sel parafol:Calcitonin Stroma amiloid20-25% ~ MEN syndrMorfologi:Tumor tersebar 1 lobusNodules kedua lobusFamilial MEN syndr10 th surv rate 90 % sedangkan MEN syndr 10 th surv rate 30-50%

  • MEDULLARY CARCINOMA