Endokrin Dr Jimmy-may2010

8/6/2019 Endokrin Dr Jimmy-may2010

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ENDOKRIN

dr. Jimmy H.W. , Sp.PA


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ENDOKRIN

Kelenjar mengeluarkan hormon


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Figure 24-1 Hormones released by the anterior pituitary. The adenohypophysis (anterior pituitary) releases five hormones that are in turnunder the control of various stimulatory and inhibitory hypothalamic releasing factors. TSH, thyroid-stimulating hormone (thyrotropin); PRL,

prolactin; ACTH, adrenocorticotrophic hormone (corticotropin); GH, growth hormone (somatotropin); FSH, follicle-stimulating hormone; LH,luteinizing hormone. The stimulatory releasing factors are TRH (thyrotropin-releasing factor), CRH (corticotropin-releasing factor), GHRH

(growth hormone-releasing factor), GnRH (gonadotropin-releasing factor). The inhibitory hypothalamic influences are comprised of PIF(prolactin inhibitory factor or dopamine) and growth hormone inhibitory factor (GIH or somatostatin).

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2005 Elsevier


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1. Molekul pemberi sinyal interaksipermukaan sel

GH, Insulin Epinefrin, Histamin

2. Steroid masuk sel membran

Estrogen, Progesteron

Glukokortikoid Tiroksin

Asam retinoat

Hormon :


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Figure 24-2 A, Photomicrograph of normal pituitary. The gland is populated by several distinct cell populationscontaining a variety of stimulating (trophic) hormones. B, Each of the hormones has different staining

characteristics, resulting in a mixture of cell types in routine histologic preparations. Immunostain for humangrowth hormone.


2005 Elsevier


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Kelenjar Hipofise ( Pituitary )

1 cm, 0,5 gr, pada sella tursica

Jenis hormon :1. Adenohipofise - GH - ACTH - FSH

- PRL - TSH - LH

2. Neurohipofise - Oksitosin

- Vasopresin

- ADH


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Figure 24-7 Homeostasis in the hypothalamus-pituitary-thyroid axis and mechanism of action of thyroid hormones. Secretion of thyroidhormones (T3 and T4) is controlled by trophic factors secreted by both the hypothalamus and the anterior pituitary. Decreased levels of

T3 and T4 stimulate the release of thyrotropin-releasing hormone (TRH) from the hypothalamus and thyroid-stimulating hormone (TSH)from the anterior pituitary, causing T3 and T4 levels to rise. Elevated T3 and T4 levels, in turn, suppress the secretion of both TRH and

TSH. This relationship is termed a negative-feedback loop. TSH binds to the TSH receptor on the thyroid follicular epithelium, whichcauses activation of G proteins, and cyclic AMP (cAMP)-mediated synthesis and release of thyroid hormones (T3 and T4). In the

periphery, T3 and T4 interact with the thyroid hormone receptor (TR) to form a hormone-receptor complex that translocates to thenucleus and binds to so-called thyroid response elements (TREs) on target genes initiating transcription.


2005 Elsevier


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Hiperpituitarism

Oleh karena :

Adenoma

Hiperplasia Carcinoma

Kelainan Hipotalamus


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ADENOMA HIPOFISE :

10 % tumor otak

Usia 30 50 tahun

Satu jenis tumor 1 jenis hormon

Makroskopis :

Batas jelas, lunak

Kecil (mm) besar (cm)

Lesi besar invasive adenoma

Perdarahan apoplexi


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Mikroskopis :

Sel uniform, poligonal, jalur-jalur/lembaran

Jaringan ikat penyangga

Inti uniform pleomorfik


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Figure 24-4 Pituitary adenoma. This massive, nonfunctional adenoma has grown far beyond the confines of thesella turcica and has distorted the overlying brain. Nonfunctional adenomas tend to be larger at the time of

diagnosis than those that secrete a hormone.


2005 Elsevier


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PROLAKTINOMA

Hiperplasi sel laktotrof karena :

Hipotalamus rusak neuron dopaminergik rusak

Obat yang menekan reseptor dopamin padahipofise

- phenotiazin

- reserpin

- haloperidol


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PROLAKTINOMA

Terapi :

Bromocriptin sebagai antagonist receptordopamin


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Somatotrof Adenoma

Mutasi somatik monoclonal

Efek tumor G H meningkat

Anak

gigantisme Dewasa acromegali

G H tinggi juga dapat karena :

- Disfungsi gonad - Arthritis

- D M - Congestive jantung

- Hipertensi - Ca Gastro intestinal


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KORTIKOTROF ADENOMA

Sering sebagai mikroadenoma

Efek ACTH naik hiperkortikoisme (penyakit

Cushing)

Nelson sindrom :

- Kortikotrof adenoma lebih besar

(setelah adrenal dioperasi)

- Hiperpigmentasi (karena MSH tinggi)


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Hipopituitarisme, karena :

Fakta Hipofise :

Tumor non fungsionil / kista

Operasi / radiasi

Apoplexi

Ischemic necrosis/post partum necrosis

(Sheehan syndrome)

Empty sella syndrome

Genetik


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Fakta Hipotalamus : Tumor primer / sekunder

Infeksi / degenerasi

Klinik hipopituitarisme :

1. Fungsi kelenjar perifer turun

Adrenal

Thyroid Gonad

2. Wajah pucat MSH rendah

3. Atrofi genitalia


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Hipofise posterior

Hormon produksi

ADH

Oksitosin

Diabetes insipidus ADH rendah

Etiologi : trauma, infeksi, tumor

Klinik : - haus

- urine banyak

- Na serum tinggi, osmositas


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Syndrome of Inappropriate ADHsecretion :

ADH tinggi Etiologi : Ca small cell paru-paru

Klinik : - urine sedikit

- Na serum rendah


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Tumor Hipotalamus

Glioma

Craniopharyngioma


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Craniopharyngioma

Dari : Vestigical Remnants Rathke Pouch

Usia : anak dewasa muda

Morfologi : Umumnya jinak

Soliter, kistik, multiloculated

Mirip adamantinoma


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Thyroid

Asal : evaginasi epitel pharyngeal

Normal : 15-20 gr

Hormon aktif : - T3,T4 bebas

- ikatan dengan

TBG


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Thyroid

fungsi :

Katabolisme : - karbohidrat

- lemak

Sintesa : - protein


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Thyroid

Goitrogen (bahan penghambat sintesa

hormon) Propil tiourasil oksidasi Jodium

Jodium pelepasan T3,T4

Jodium dosis tinggi proteolisis tiroglobulin


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Hipertiroidisme

Tirotoxicosis dapat karena :

Diffuse hiperplasi (85% Graves)

hipertiroidisme Tx hormon tiroid berlebihan

Multinodular goiter

Neoplasma tiroid Tiroiditis


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Hipertiroidisme

Terjadi :1. Hipermetabolik

2. Overaktif simpatetik


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Hipertiroidisme

Gejala Hipertiroid :

1. Cardiac : aritmi/palpitasi/cardiomegali

2. Otot : atrofi / fatty changes

3. Tulang : osteoporose, fraktur

4. Limfoid : hiperplasi


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Hipertiroidisme

5. Ocular : Staring gaze, lid lag

6. Neuromuscular : tremor, cemas,

insomnia, emosional7. Kulit : berkeringat, rasa panas,

kemerahan

8. GI : rasa haus, lapar


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Hipertiroidisme

Dx :

o Tanda klinik

o Lab : - T4 bebas >>

- T S H


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Hipotiroidi

Sebab :

1. Primer gangguan tiroid

2. Sekunder


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Hipotiroidi

Hipotiroidi karena parenchim tiroid :

Embrional

Radiasi

Operatif

Hashimoto


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Hipotiroidi

Hipotiroidi karena sintesa :

Idiopatik

Cacat sintesa turunan

Jodium intake kurang

Bahan-bahan goitrogen


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Hipotiroidi

Hipotiroidi karena supratiroidal :

Lesi hipofise

Lesi hipotalamus


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Cretinisme

Ciri klinik :

Pertumbuhan skeletal dan syaraf

Antara lain :

Retardasi mental

Tubuh pendek, kecil

Wajah kasar

Lidah menjulur

Hernia umbilicus


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Mixedema

Pada anak besar dewasa

Aktifitas fisik dan mental

Tanda-tanda klinik :- fatique

- apatis

- bicara & intelektual

- tidak tahan dingin


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Mixedema

Tanda-tanda klinik :

- gemuk

- simpatetik : konstipasi, kulitkering/dingin/pucat

- cardiac output : napas pendek

- tidak tahan dingin


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Mixedema

P A :

- matrix glycosaminoglycan

- matrix hyaluronicacid

Lab :

- T4 , T3

- T S H

seluruh

jaringan


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Patogenesis

Terdapat HLA DR3, HLA DR 5

Defect T cell mengenai MHC Ag

T cell suppressor T cell helperCD8+ cytotoxic

Ab B cell epitel rusak


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Figure 24-9 Pathogenesis of Hashimoto thyroiditis. Three proposed models for mechanism of thyrocyte destruction in Hashimoto disease. Sensitization of autoreactive

CD4+ T cells to thyroid antigens appears to be the initiating event for all three mechanisms of thyroid cell death. See the text for details.


2005 Elsevier


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Ab yang timbul

Ab Tiroglobulin

Ab Tiroperoxidase

Ab TSH rec menghambat T3 T4

Ab Jodium transporter


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Morfologi

Diffuse, berbatas jelas

Pucat, abu-abu, kenyal, noduler

Kapsul intak


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Klinik

Struma tidak nyeri, simetrik diffuse

Kadang-kadang noduler

Hipotiroidisme, kadang-kadanghipertiroidisme transien

Risk factor timbul limfoma


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Figure 24-9 Pathogenesis of Hashimoto thyroiditis. Three proposed models for mechanism of thyrocyte destruction in Hashimoto disease. Sensitization of autoreactive

CD4+ T cells to thyroid antigens appears to be the initiating event for all three mechanisms of thyroid cell death. See the text for details.


2005 Elsevier


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De Quervain Tiroiditis :

Jarang terjadi

Usia 30 50 tahun Wanita : Pria = 3 5 : 1


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Ada faktor infeksi virus, seperti :

- coxsachie virus - measles

- mumps - adenovirus

Ada HLA B 35virus

jaringan rusak

Sel rusak cytotoxic T cell

Ag makrofag (HLA B35)

Patogenesis :


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Morfologi :

Unilateral / bilateral

Kenyal, kapsul intak

Kadang-kadang perlekatan jaringan

sekitar

Warna kuning pucat, kecoklatan


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Klinik :

Terjadi mendadak / bertahap

Nyeri leher, panas, capek, malas, anorexi,myalgin

Terdapat struma

Dapat sembuh spontan

T3 T4 , TSH


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Graves Disease

Triad yang harus ada :

1. Hipertiroidi, dengan goiter aktif

2. Ophthalmopathy exophthalmos

3. Dermatopathy pretibial myxedema


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Klinik :

oU

sia 20-40 tahun, wanita : pria=

7 : 1o Ada faktor genetik

o Terdapat MHC - haplotype HLA DR3

HLA DR8


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Figure 24-8 A patient with hyperthyroidism. A wide-eyed, staring gaze, caused by overactivit y of the sympathetic nervous system, is one of the features of this disorder.

In Graves disease, one of the most important causes of hyperthyroidism, accumulation of loose connective tissue behind the eyeballs also adds to the protuberant

appearance of the eyes.


2005 Elsevier

P t i


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Patogenesis

Terdapat Ab :

1. TSI = Thyroid stimulating immnuoglobulin

2. TGI = Thyroid growth stimulating

immnuoglobulin

3. TSH binding inhibitor immunoglobulin (

antibody anti TSH receptor )


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Figure 24-12 Diffusely hyperplastic thyroid in a case of Graves disease. The follicles are lined by tall, columnar epithelium. The crowded,enlarged epithelial cells project into the lumens of the follicles. These cells actively resorb the colloid in the centers of the follicles,

resulting in the scalloped appearance of the edges of the colloid.


2005 Elsevier


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1. Molecular mimicry :

Proses infeksi

Bahan2 exogen

2. Primary T cell autoantibody :

MHC Ag HLA DR gene protein Ag

Ab B cell T cell active

prosesimunitas

Ab

Teori terjadinya Graves :


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Ophthalmopathy graves

Jaringan ikat orbita

Otot extra ocular

Ab B cell T cell, CD4+ / CD8+

Mirip TSH receptor


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Morfologi :

Struma, Sp 80 gr, simetri berkapsul Konsistensi lunak, halus, merah seperti daging

Sel-sel silindris, papil-papil kecil

Colloid sedikit, dengan scalloped margin Infiltrasi limfosit ( B cell )


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Terapi :

Jodium involusi epitel

sekresi tiroglobulin turun Propilthiouracil sintesa kurang

Radioaktif jodium

pembedahan


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Diffuse Non Toxic ( simple ) Goiter

a. Endemic

Pada semua usia terutama muda

Pada daerah rendah jodium

pegunungan 10 % populasi (+)

Ada faktor goitrogen :

- calcium - lobak

- kubis - singkong

- bloomkol


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b. Sporadik

Wanita > pria

Usia pubertas dewasa muda

Terdapat perubahan kelainan pada : -transport jodium

- dehalogenase

- organification

- iodotyrosin coupling


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Multinodular goiter

Dapat sporadik atau endemik

Usia lebih tua

Menyerupai neoplasm


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Patogenesis

TSH TiroidHiperplasi

Involusi colloid Folikel besar

pecahPer-

darahanfibrosis

kalsifikasiNodul2

bergabung

Multinodulargoiter


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Morfologi :

Multilobulated sp 2.000 gr Asimetrik, masuk ke substernal plunging

goiter

Bila 1 nodul dominan solitary nodule(adenomatous goiter)

Irisan warna coklat, gelatinous, fibrosis,

perdarahan, kalsifikasi, kistik

Mikroskopis : folikel banyak colloid, epitel pipih,

atrofik/hiperplasi

Kli ik


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Klinik :

Bila besar gangguan kosmetik

tekanan pada trachea,oesophagus, vena2

Bila salah satu nodulnya hiperfungsi Plummer Syndrome


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Figure 24-13 Nodular goiter. The gland is coarsely nodular and contains areas of fibrosis and cystic change.


2005 Elsevier


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B b


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Beberapa kriteria penyokong Dx

Nodul soliter neoplasma

Usia muda neoplasma

Jenis kelamin laki-laki neoplasma Pernah diterapi R Ca

Hot nodule jinak


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Adenoma tiroid

Soliter

Folikel follicular adenoma

Beberapa jenis, tersering : simplecolloid adenoma

Adenoma sangat jarang menjadiCarcinoma


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Figure 24-14 Follicular adenoma of the thyroid. A solitary, well-circumscribed nodule is seen.

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Patogenesis

TSH receptor merupakan keluarga 7anggota transmembrane kelompok

protein G receptor TSH rec subunit stimulating guanine

nucleotide binding protein Gs adenylcyclose C AMP gene aktif hormon ,

proliferasi


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Patogenesis

Mutasi somatik stimulasi C AMP kronik sel tumbuh lebih cepat autonomously

functioning monoclonal adenoma


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Morfologi adenoma

Tumor soliter

Bentuk speris

Berkapsul, tekanan jaringan sekitar Ukuran sekitar 3 cm

Warna abu-abu putih, merah kecoklatan

Kadang

2

perdarahan, fibrosis, kalsifikasi,kistik


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mikroskopis

Folikel ukuran sama, isi folikel Jenis : - Simple colloid (macrofolicular)

- Fetal (microfolicular)- Embryonal (trabecular)- Hurthle cell (oxiphyl, oncocyte)- Atypical

- Adenoma with papillae(=Papillae adenoma)(=EncapsuledPapillary Ca)

T Ti id Ji k l i


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Tumor Tiroid Jinak yang lain

Kista tiroid : - deri adenoma folicular

- dari multinodular

goiter Kista dermoid

Lipoma

Hemangioma Terratoma

Carcinoma Thyroid


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Carcinoma Thyroid

Umumnya usia dewasa

Wanita > pria, khususnya usia muda

Terdapat reseptor estrogen pada sel-seltumor

Jenis Carcinoma


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Jenis Carcinoma

Papillary Ca 75-85 %

Follicular Ca 10-30 %

Medullary Ca 5 %

Anaplastic Ca 5 %

Patogenesis


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Patogenesis

Faktor radiasi terutama usia dibawah 20

th

Adanya colloid goiter adan Thyroiditis Terjadinya aktivasi/mutasi RET oncogen

terutama papillary & medullary Carcinoma

Patogenesis


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Patogenesis

Mutasi RAS gene terjadi pada adenoma

dan carcinoma

Mutasi inaktivasi pada gene tumorsuppressor P53 khususnya pada

anaplastic carcinoma

Papillary Carcinoma


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Papillary Carcinoma

Semua usia, terutama 20-40 tahun

Erat hubungannya dengan fakta radiasi


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Figure 24-17 Papillary carcinoma of the thyroid. A, The macroscopic appearance of a papillary carcinoma with grossly discernible papillary structures. This particular

example contains well-formed papillae (B), lined by cells with characteristic empty-appearing nuclei, sometimes termed " Orphan Annie eye" nuclei (C). D, Cells obtained

by fine-needle aspiration of a papillary carcinoma. Characteristic intranuclear inclusions are visible in some of the aspirated cells.



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Figure 24-17 Papillary carcinoma of the thyroid. A, The macroscopic appearance of a papillary carcinoma with grossly discernible papillary structures. This particular

example contains well-formed papillae (B), lined by cells with characteristic empty-appearing nuclei, sometimes termed " Orphan Annie eye" nuclei (C). D, Cells obtained

by fine-needle aspiration of a papillary carcinoma. Characteristic intranuclear inclusions are visible in some of the aspirated cells.


Morfologi


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Morfologi

Soliter atau multipel

Berbatas jelas / berkapsul / menyebardiluarnya

Kadang2 fibrosis, kalsifikasi, kistik

Pada irisan granula / papil-papilkecil

Mikroskopis :


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Mikroskopis :

Papil dengan fibrovasculer, dilapisiepitel

Inti dengan ground glass / orphanannie

Intra nuclear inclusion / groves

Psammoma bodies

Klinik


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Klinik

Sering a symptomatic

Sering dengan metastasis kelenjarleher

Radioactive jodium cold nodule

FNA, cara Diagnosa yang tepat


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Morfologi : Single nodule

Batas jelas / infiltratif Tumor besar infiltrasi ke jaringan

sekitar

Warna abu-abu coklat merah muda

Kadang2 fibrosis, kalsifikasi


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Figure 24-18 Follicular carcinoma. Cut surface of a follicular carcinoma with substantial replacement of the lobe of the thyroid. The tumor has a light-tan appearance and

contains small foci of hemorrhage.



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Figure 24-19 Follicular carcinoma of the thyroid. A few of the glandular lumens contain recognizable colloid.

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Klinik :

Nodul kecil, lambat laun membesar

R cold nodule

Metastasis hematogen ke organ-organ

jauh

Medullary Carcinoma


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Medullary Carcinoma

Dari para follicular cell

Hormon yang dikeluarkan-Calcitonin - Serotonin

-CEA - Somatostatin

-VIP (Vasoactive IntestinalPeptide)

Medullary Carcinoma


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Medullary Carcinoma

80% type sporadic

20% a. termasuk MEN syndrome

b. familial

Sporadic & familial dewasa 40-50tahun

MEN syndrome anak-anak


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Figure 24-21 Medullary carcinoma of thyroid. These tumors typically show a solid pattern of growth and do not have connective tissue capsules. (Courtesy of Dr. Joseph

Corson, Brigham and Women's Hospital, Boston, MA.)

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Figure 24-21 Medullary carcinoma of thyroid. These tumors typically show a solid pattern of growth and do not have connective tissue capsules. (Courtesy of Dr. Joseph

Corson, Brigham and Women's Hospital, Boston, MA.)

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Morfologi


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Morfologi

Soliter type sporadic

Multipel type familial

Jaringan tumor halus, warna abu2-coklat

Kadang2 nekrosis, perdarahan

Klinis


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Klinis

Nodul di thyroid

Kadang2 disertai diare karena VIP

Type sporadic / MEN tumbuhagresif

Type familial low grade

Mikroskopis


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Mikroskopis

Sel poligonal, spindle, dalamsarang/trabekula/folikel

Deposit amiloid ( dari molekulcalcitonin)

Anaplastic Carcinoma :


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Anaplastic Carcinoma :

Sangat agresif

Usia tua, 65 tahun

Sering didahului multinodular goiter

Morfologi :


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Morfologi :

Large, pleomorfik giant cell

Spindle cell

Small anaplastic cell

Congenital anomali Tiroid


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Congenital anomali Tiroid

Ductus/cyst thyroglossus

Sisa2 vestigial remnant

Lesi kecil 2-3 cm Letak antara Glossus - Thyroid

Parathyroid


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Parathyroid

Dari kantung pharyngeal, ada 4 kelenjar

Berat 35-40 mg

Terdiri dari - chief cell germal parathormon

- oxyphil cell

Kerja parathyroid dikendalikan oleh Ca ion darah


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Figure 24-24 Parathyroid adenomas are almost always solitary lesions. Technetium-99m-sestamibi radionuclide scan demonstrates an area of increased uptake

corresponding to the left inferior parathyroid gland (arrow). This patient had a parathyroid adenoma. Preoperative scintigraphy is useful in localizing and distinguishing

adenomas from parathyroid hyperplasia, where more than one gland would demonstrate increased uptake.


2005 Elsevier


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Figure 24-25 Parathyroid adenoma. A, Solitary chief cell parathyroid adenoma (low-power photomicrograph) revealing clear delineation from the residual gland below. B,

High-power detail of a chief cell parathyroid adenoma. There is some slight variation in nuclear size but no anaplasia and some slight tendency to follicular formation.


2005 Elsevier

Hormon PTH bekerja :


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j

a. Pada tulang menambah aktivitasosteoclast

b. Pada ginjal meningkatkan

- resorbsi calcium

- konversi vit D aktif

- ekskresi phosphat

c. Pada usus menambah absorbsi kalsium


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Hiperparatiroidisme primer


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Hiperparatiroidisme primer

Sebabnya :

Adenoma 75-80 %

Hiperplasia 10-15 % Carcinoma 5 %

Usia tersering pada dewasa 50 th lebih

Wanita lebih sering dp laki-laki Ada faktor radiasi sebelumnya


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Figure 24-26 Cardinal features of hyperparathyroidism. With routine evaluation of calcium levels in most patients,primary hyperparathyroidism is often detected at a clinically silent stage. Hypercalcemia from any other cause can

also give rise to the same symptoms.


2005 Elsevier


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Pada symptomatic timbul :

Tulang osteoporosis

Ginjal nephrolithiasis

Gastrointestinal constipasi, ulcus dll

CNS depresi

Neuromuscular lemah

Cardiac kalsifikasi katup

Adrenal Cortex :


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Adrenal gland cortex

medulla

Adrenal cortex :

1. Glucocorticoid (cortisol)

2. Mineralocorticoid (aldosteron)

3. Sex steroid (estrogen/androgen)

Adrenal medulla catecholamine(epinephrin)


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Figure 24-47 Nodular hyperplasia of the adrenal contrasted with normal adrenal gland. In cross-section, the adrenal cortex is yellow,thickened, and multinodular, owing to hypertrophy and hyperplasia of the lipid-rich zonae fasciculata and reticularis.


2005 Elsevier


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Figure 24-48 Consequences of C-21 hydroxylase deficiency. 21-Hydroxylase deficiency impairs

the synthesis of both cortisol and aldosterone. The resultant decrease in feedback inhibition

(dashed line) causes increased secretion of adrenocorticotropic hormone, resulting ultimately in

adrenal hyperplasia and increased synthesis of testosterone. The sites of action of 11-, 17-, and

21-hydroxylase are shown by the numbers in circles.Downloaded from: Robbins & Cotran Pathologic BasisofDisease (on 14 April2005 04:00 AM)

2005 Elsevier

Adrenocorticol hyperfunction(hyperadrenalism) :


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(hyperadrenalism) :

1. Cushing syndrome

2. Hyperaldosteronism

3. Adrenogenital syndrome

Cushing syndrome


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Produk glucocortikoid

Sebab2 glucocorticoid tinggi :

1. Pemberian obat2 glucocorticoid luar(iatrogenic)

2. Primary hypothalamic pituitary disease

3. Hypersecretion of cortisol in adrenal

4. Secretion of ectopic ACTH in non endocrineneoplasm


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Figure 24-43 A schematic representation of the various forms of Cushing syndrome, illustrating the three endogenous forms as well asthe more common exogenous (iatrogenic) form. ACTH, adrenocorticotropic hormone.


2005 Elsevier


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Figure 24-44 The major causes of primary hyperaldosteronism and its principal effects on the kidney.


2005 Elsevier

Pituitary Cushingsyndrome (50%)


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Adenoma hipofise

ACTH Hyperplasia hipofise karena trofik hormon

hipofise

ciri : - cortisol

- ACTH

AdrenalCushingsyndrome (15-30%)

Adenoma / carcinoma adrenal Hyperplasia adrenal

ciri : - cortisol

- ACTH

ParaneoplasticCushingsyndrome


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a a eop ast c Cus g sy d o e

Small cel Ca paru Carcinoid tumor

Medullary Ca thyroid

Islet cell Ca pancreas

ciri : - cortisol - ACTH (karena corticotropin releasing

factor dari tumor)

Morfologi

Jaringan hipofise & adrenal kondisinyatergantung jenis kelainannya


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Figure 24-45 Adrenal cortical adenoma. The adenoma is distinguished from nodular hyperplasia by its solitary, circumscribed nature. Thefunctional status of an adrenal cortical adenoma cannot be predicted from its gross or microscopic appearance.


2005 Elsevier


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Figure 24-46 Histologic features of an adrenal cortical adenoma. The neoplastic cells are vacuolated because of the presence ofintracytoplasmic lipid. There is mild nuclear pleomorphism. Mitotic activity and necrosis are not seen.


2005 Elsevier

Insufisiensi Adrenocortical


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1. Primary acute adrenocortical insufficiency(adrenal crysis)

2. Primary chronic adrenocortical insufficiency(Addison Disease)

3. Secondary adrenocortical insufficiency

Adrenal crysis


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Chronic adrenocortical insufficiency KU pasien tambah parah

Tx corticosteroid withdrawl

Adrenal hemorrhage : Bayi lahir dg masalah

Tx anticoagulant

DIC Bacteremia

Waterhouse FriderichsenSyndrome anak2


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Syndrome anak

Bacterial infection :

Neisseria meningitis

Pseudomonas Haemofilus influinza

Hypotensi shock

DIC


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Figure 24-49 Waterhouse-Friderichsen syndrome in a child. The dark, hemorrhagic adrenal glands are distendedwith blood.


2005 Elsevier


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Figure 24-50 Waterhouse-Friderichsen syndrome. At autopsy, the adrenals were grossly hemorrhagic and shrunken; microscopically, little residual cortical architecture is

discernible.


2005 Elsevier

Friderichsen Syndrome


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Adrenocortical insuff karena

Adrenal haemorrhage dan bacterial infection

Ciri-ciri :

Infeksi karena - neisseria meningitis

- pseudomonas

- Pneumococci

- haemophilus influenza Hipotensi progresif shock


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Addison disease


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= Primary chronic adrenocortical insufficiency

Gejala klinik timbul bila lebih 90 % adrenalrusak

Patogenesis

90% karena autoimune adrenalitis, TBC,metastasis Ca

1. Autoimmune adrenalitis (60-70%)


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Selain adrenal organ lain sering ikutterjadi proses autoimmune tsb, yaitupada

Hashimoto Pernicious anemia

DM type II

Idiopathic hypoparathyroidism

Didapatkan antiadrenal antibodies

Didapatkan HLA B8 DR 3

2. infection


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Sering karena TBC 90% Addison (dulu)

3.Metastatic Carcinoma

Ca dari paru-paru, mamma dll


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Figure 24-52 Adrenal carcinoma. The hemorrhagic and necrotic tumor dwarfs the kidney and compresses the upper pole.


2005 Elsevier


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Figure 24-52 Adrenal carcinoma. The hemorrhagic and necrotic tumor dwarfs the kidney and compresses the upper pole.


2005 Elsevier

Medulla Adrenalis


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Sel chromaffin catecholamines (epinephrin,

norepinephrin)

Sel ini diinduksi oleh serat syaraf preganglion

sistem sympathetic Norepinephrin : = adrenalin, pada sirkulasi aliran

darah E/F adrenergic receptor sel > aktif (mis:

myocard , vasoconstricsi )


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Sel chromaffin adalah termasuk sel

neuroendocrin (epinephrin, norepinephrin)

Sel neuroendocrin (diluar Medulla adrenalis)

membentuk kelompok2 /nodul paraganglion

sistem ini termasuk sistem syaraf otonomic ada 3 :

- bronchiomeric

- intravasal

- aortico sympathetic

Pheochromocytoma


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Dari sel chromaffin catecholamin

85% timbul pada medulla, yang lain dariparaganglion extra adrenal

90% sporadic

10% autosomal dominant, familialsyndrome

Pheochromocytoma


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Type sporadic usia 40-60 th

Wanita sedikit lebih banyak

Type familial

anak-anak, laki-laki > Dx ganas hanya ditentukan metastasis +/-


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Klinik :

Tanda utama hipertensi

Nyeri dada, nusea, vomiting Pada jantung catecholamin

cardiomyopathy


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Figure 24-55 Pheochromocytoma. The tumor is enclosed within an attenuated cortex and demonstrates areas of hemorrhage. Thecomma-shaped residual adrenal is seen below.


2005 Elsevier


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Figure 24-55 Pheochromocytoma. The tumor is enclosed within an attenuated cortex and demonstrates areas of hemorrhage. The comma-shaped residual adrenal is

seen below.


2005 Elsevier

Neuroblastoma


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Lokasi :

o Adrenal medulla

o Tempat lain : sistem syaraf sympathetic

Pineal


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Pine cone shaped

100 180 gr

Tumor pinealoma


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Endokrin Dr Jimmy-may2010

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