Tumor Urogenital

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Dr. Resti Arania Sp.PA

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Tumor Urogenital

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TUMOR TRACTUS UROGENITALDr. Resti Arania Sp.PA

WILMS' TUMOR

= Nephroblastoma = , umur median 2 th 11 blnKa = Ki, 5 % bilateral15 % + kelainan kongenital : Anomali UG Hemihipertrofia Aniridia

ETIOLOGI : Diduga kongenitalKLINIS : Flank mass Flank pain Hematuria Hipertensi Anorexia, Nausea, Vomiting Kelainan kongenital lainLABORATORIUM : Hematuri AnemiaTriasRADIOLOGIS :

BNO : - Kesuraman salah satu sisi perut - Usus terdesak oleh massa

IVP: Collecting sytem terdesak massa / distorsi

USG: Massa padat dalam ginjalPATOLOGI :Campuran Epithelial, Stromal, Blastematous( Immature Mesenchyma )

2 kelompok :- Favorable histology89 %- Unfavorable H 11 %

Prognosa kurang baikSTAGING ( Menurut NWTS ) :

Terbatas dalam ginjal, eksisi sempurna

Keluar ginjal, eksisi sempurna

Sisa tumor dalam abdomen

Metastase jauh

BilateralDD: Neuroblastoma Teratoma Hamartoma Hidronefrosis Cystic kidneysTERAPI : Radical nephrectomy Chemotherapy : vincristine + Actinomycin D ( adriamycine) Radiasi PROGNOSAStage2Y Relapse free2 YSR I II III IV V 88 %78 %70 %49 %95 %90 %84 %54 %87 %GRAWITZ' TUMOR

Renal cell Ca Adeno Ca ginjal Hypernephroma = Clear cell Ca

Pria : Wanita = 2 : 1Sering pada dekade 5 -6

Penyebab ?Faktor resiko : - merokok - analgesik - dllTanda dan Gejala :Trias :- Gross hematuria- Flank pain- Flank massLaboratorium : - Hematuria- Anemia- LED Radiologi :- IVP: distorsi PCS- USG: massa di ginjal- CT Scan: massa di ginjal

Terapi : - Nefrektomi Radikal

Terapi Ajuvan :- Radiasi- Hormonal- Kemoterapi

TUMOR BULI-BULI

Tumor yang tumbuh dari epitel buli-buliJenis: - Transitional cell Ca90 % - Epidermoid Ca/ KSS5 - 10 % - Adeno Ca2 %

Pria : Wanita = 2,7 : 115Penyebab: Belum jelas

Faktor resiko : Merokok Pekerja yang berhubungan dengan ; Bahan kimia Cat Karet Bensin Kulit Trauma fisik : Infeksi Instrumentasi BatuTanda dan Gejala : Painless, gross hematuria 90 % Polakisuria, dysuria, urgency Nyeri tulang, nyeri pinggang Massa supra pubisDiagnosis : Urinalysis : hematuria Sitologi urine : klas IV - V IVP : filling defect dalam buli-buliKomplikasi : Anemia Gagal ginjal kronisPenatalaksanaan : TUR Buli Sistektomi partial Sistektomi total Kemoterapi intravesikal Radiasi Kemoterapi

Overview of organogenesis of the urogenital organsUrinary and reproductive systems are closely associated in topography,function and development. Two systems have common origin from the urogenital ridge(UGR) and have homologous structures. Internal genital duct system is derived from the foetal urinary system. Malformation of one system affects the other.The UGR is longitudinal swelling in dorsolateral side of the abdomenUGR--> formed mostly from--non-segmented intermediate mesodermLateral UGR(nephrogenic plate) forms urinary organs and internal genital ducts. Ventromedial UGR is genital ridge, forms gonads.

The Urogenital ridgeMesonephricDuctParamesonephric ductGonadCr.VDCa.(genital ridge)Mesonephros(nephrogenicPlate) SEX DETERMINATIONGonadal ridgeBipotential gonadOvaryTestisOestrogenMullerian/ParamesonephricductFemale genitalducts Anti-Mullerian hormone(Sertoli cells)Testosterone(Leydig cells)Regression of MullerianductDifferentiation of Wolffian duct intoMale genital ductsDescent of testisSRYDAXWnt 4Red arrows shows gene regulationPrimary sex determination at fertilisationgenetic sex: XY, XX(ii).Y chromosomes encodes testis-determining factorSRYgonadogenesis secretion of foetal hormones by interstitial cells[(Sertoli and Leydig(M),theca cells(F)] secondary sex(phenotypic sex)

Gonadogenesis occurs at the genital ridge initiated by 2 simultaneous events:-(i).Formation of gonadal cordsEpithelium from degenerate mesonephric nephrons invade genital ridge.And form network of epithelial cords(ii).Migration of primordial germ cells.PGC are endodermal cells, migrate from the yolk sac into the bipotential gonad.The gonad has a central medulla and a peripheral cortex, surrounded by coelomic epithelium.

Gonadogenesis(2). The bipotential gonadDorsal aortaArterioleGlomerulusMesonephrictubuleGenitalridgeMesonephric/WolffianductDorsalmesenteryMigratiing PGCPGC arrive at the genital ridge at 21 days cat and proliferate.Epithelial incorporate PGC, forming gonadal cords. Gonadal differentiation begins.

PROSTATE NEOPLASIABENIGN PROSTATIC HYPERPLASIAANDPROSTATE CANCER24PROSTATE ANATOMYfibromuscular tissue (30-50%)glandular epithelial cells (50-70%)peripheral zone (most cancers)central zonetransition zone (BPH,low grade cancers)

BENIGN PROSTATIC HYPERPLASIA

17% of men age 50-59 (require Rx)27% of men age 60-69 (require Rx)35% of men age 70-79 (require Rx)Similar crosscultural prevalenceSome genetic and racial susceptibility to symptom severity (autosomal dominant)Diet high in saturated fats, zinc and low in fruits and vegetables.Sedentary life style.

BPHProposed EtiologiesReawakening of the urogenital sinus to proliferateChange in hormonal milieu with alterations in the testosterone/estrogen balanceInduction of prostatic growth factorsIncreased stem cells/decreased stromal cell death

BPHPathophysiologySlow and insidious changes over timeComplex interactions between prostatic urethral resistance, intravesical pressure, detrussor functionality, neurologic integrity, and general physical health.

BPH PathophysiologyInitial hypertrophydetrussor decompensationpoor tonediverticula formationincreasing urine volumehydronephrosisupper tract dysfunction

PENIS KARSINOMA

TESTICULAR TUMOUR1% of all Malignant TumourAffects young adults - 20 to 40 yrs - when Testosterone Fluctuations are maximum90% to 95% of all Testicular tumours from germ cells 99% of all Testicular Tumours are malignant. Causes Psychological & Fertility Problems in young

Survival in Testicular TumoursImproved overall survival in last 15 to 20 years due to - Better understanding of Natural History and Pathogenesis of diseaseReliable Tumour Markers Cis-platinum based chemotherapy

39CROSS SECTION OF TESTIS Testis

StromaSeminiferous Tubules (200 to 350 tubules)

Interstitial Cells Supporting SpermatogoniaLeydig or(Androgen) Sertoli CellEPIDEMIOLOGYIncidence :1.2 per 100,000 (Bombay)3.7 per 100,000 (USA)Age :3 Peaks- 20-40 yrs. Maximum- 0 - 10 yrs.- After - 60 yrs.Bilaterality : 2 to 3% Testicular Tumour CLASSIFICATIONI.Primary Neoplasma of Testis.A.Germ Cell Tumour B.Non-Germ Cell Tumour

II.Secondary Neoplasms.

III.Paratesticular Tumours.I. PRIMARY NEOPLASMS OF TESTISA.Germinal Neoplasms : (90 - 95 %)1.Seminomas - 40%(a)Classic Typical Seminoma(b)Anaplastic Seminoma(c)Spermatocytic Seminoma2.Embryonal Carcinoma - 20 - 25%3.Teratoma - 25 - 35%(a)Mature(b)Immature4.Choriocarcinoma - 1%5.Yolk Sac Tumour

I. PRIMARY NEOPLASMS OF TESTISB.Nongerminal Neoplasms : ( 5 to 10% )1.Specialized gonadal stromal tumor(a)Leydig cell tumor(b)Other gonadal stromal tumor2.Gonadoblastoma3.Miscellaneous Neoplasms(a)Adenocarcinoma of the rete testis(b)Mesenchymal neoplasms(c)Carcinoid(d)Adrenal rest tumor

A.AdenomatoidB.Cystadenoma of EpididymisC.Mesenchymal NeoplasmsD.MesotheliomaE.MetastasesII. SECONDARY NEOPLASMS OF TESTISA.Reticuloendothelial NeoplasmsB.MetastasesIII.PARATESTICULAR NEOPLASMSAETIOLOGY OF TESTICULAR TUMOUR1.Cryptorchidism

2.Carcinoma in situ

3.Trauma

4.AtrophyCRYPTORCHIDISM & TESTICULAR TUMOURRisk of Carcinoma developing in undescended testis is

14 to 48 times the normal expected incidence CRYPTORCHIDISM & TESTICULAR TUMOURThe cause for malignancy are as follows:Abnormal Germ Cell MorphologyElevated temperature in abdomen & Inguinal region as opposed to scrotumEndocrinal disturbancesGonadal dysgenesis

To properly Stage Testicular Tumours following are pre-requisites: (a)Pathology of Tumour Specimen(b)History(c)Clinical Examination(d)Radiological procedure - USG / CT / MRI / Bone Scan(e)Tumour Markers - HCG, AFPRequirements for staging Investigation1.Ultrasound - Hypoechoic area2.Chest X-Ray - PA and lateral views3.CT Scan4.Tumour Markers- AFP- HCG- LDH- PLAPCLINICAL FEATURESPainless Swelling of One GonadDull Ache or Heaviness in Lower Abdomen10% - Acute Scrotal Pain10% - Present with Metatstasis- Neck Mass / Cough / Anorexia / Vomiting / Back Ache/ Lower limb swelling5% - GynecomastiaRarely - Infertility

Tumour MarkersTWO MAIN CLASSESOnco-fetal Substances : AFP & HCGCellular Enzymes : LDH & PLAP ( AFP - Trophoblastic Cells HCG - Syncytiotrophoblastic Cells )

AFP ( Alfafetoprotein )NORMAL VALUE: Below 16 ngm / mlHALF LIFE OF AFP 5 and 7 days

Raised AFP : Pure embryonal carcinomaTeratocarcinoma Yolk sac Tumour Combined Tumour

REMEMBER: AFP Not raised is Pure Choriocarcinoma or Pure SeminomaHCG ( Human Chorionic Gonadotropin )Has and polypeptide chain

NORMAL VALUE: < 1 ng / ml HALF LIFE of HCG: 24 to 36 hours

RAISED HCG - 100 % - Choriocarcinoma 60% - Embryonal carcinoma 55% - Teratocarcinoma\25% - Yolk Cell Tumour7% - SeminomasROLE OF TUMOUR MARKERSHelps in Diagnosis - 80 to 85% of Testicular Tumours have Positive Markers Most of Non-Seminomas have raised markersOnly 10 to 15% Non-Seminomas have normal marker level After Orchidectomy if Markers Elevated means Residual Disease or Stage II or III DiseaseElevation of Markers after Lymphadenectomy means a STAGE III Disease

ROLE OF TUMOUR MARKERS cont...Degree of Marker Elevation Appears to be Directly Proportional to Tumour BurdenMarkers indicate Histology of Tumour: If AFP elevated in Seminoma - Means Tumour has Non-Seminomatous elementsNegative Tumour Markers becoming positive on follow up usually indicates -Recurrence of TumourMarkers become Positive earlier than X-Ray studies

PRINCIPLES OF TREATMENT

Treatment should be aimed at one stage above the clinical stage Seminomas - Radio-Sensitive. Treat with Radiotherapy.Non-Seminomas are Radio-Resistant and best treated by SurgeryAdvanced Disease or Metastasis - Responds well to Chemotherapy CONCLUSIONImproved Overall Survival of Testicular Tumour due to Better Understanding of the Disease, Tumour Markers and Cis-platinum based ChemotherapyCurrent Emphasis is on Diminishing overall Morbidity of Various Treatment Modalities

SEMINOMA

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