Dr. Resti Arania Sp.

PA
WILMS' TUMOR

= Nephroblastoma
 = , umur median 2 th 11 bln
Ka = Ki, 5 % bilateral
15 % + kelainan kongenital :
• Anomali UG
• Hemihipertrofia
• Aniridia

ETIOLOGI : Diduga kongenitall

KLINIS :
• Flank mass
• Flank pain
• Hematuria Trias
• Hipertensi
• Anorexia, Nausea, Vomiting
• Kelainan kongenital lain

LABORATORIUM :
• Hematuri
• Anemia
RADIOLOGIS :

• BNO : - Kesuraman salah satu sisi perut

- Usus terdesak oleh massa

• IVP : Collecting sytem terdesak massa /

distorsi

• USG : Massa padat dalam ginjal

PATOLOGI :
Campuran Epithelial, Stromal, Blastematous
( Immature Mesenchyma )

2 kelompok :
- Favorable histology 89 %
- Unfavorable H 11 %

Prognosa kurang baik

STAGING :

I. Terbatas dalam ginjal, eksisi sempurna

II. Keluar ginjal, eksisi sempurna

III. Sisa tumor dalam abdomen

IV. Metastase jauh

V. Bilateral
DD :
• Neuroblastoma
• Teratoma
• Hamartoma
• Hidronefrosis
• Cystic kidneys

TERAPI :
• Radical nephrectomy
• Chemotherapy : vincristine + Actinomycin D
( adriamycine)
• Radiasi
 PROGNOSA

Stage 2Y Relapse free 2 YSR

I 88 % 95 %
II 78 % 90 %
III 70 % 84 %
IV 49 % 54 %
V 87 %
GRAWITZ' TUMOR

• Renal cell Ca
• Adeno Ca ginjal
• Hypernephroma = Clear cell Ca

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

Penyebab ?
Faktor resiko : - merokok
- analgesik
- dll
Tanda dan Gejala :
Trias : - Gross hematuria
- Flank pain
- Flank mass

Laboratorium :
- 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-buli

Jenis : - Transitional cell Ca 90 %
- Epidermoid Ca/ KSS 5 - 10 %
- Adeno Ca 2%

Pria : Wanita = 2,7 : 1

Penyebab : Belum jelas

Faktor resiko :
• Merokok
• Pekerja yang berhubungan dengan ;
 Bahan kimia
 Cat
 Karet
 Bensin
 Kulit
• Trauma fisik :
 Infeksi
 Instrumentasi
 Batu
Tanda dan Gejala :

 Painless, gross hematuria 90 %

 Polakisuria, dysuria, urgency

 Nyeri tulang, nyeri pinggang

 Massa supra pubis

Diagnosis :
• Urinalysis : hematuria
• Sitologi urine : sel atipik ?
• IVP : :g defect dalam buli-buli

Komplikasi :
• Anemia
• Gagal ginjal kronis
Penatalaksanaan :
• TUR Buli
• Sistektomi partial
• Sistektomi total
• Kemoterapi intravesikal
• Radiasi
• Kemoterapi
 Overview of organogenesis of the urogenital organs
Urinary and reproductive systems Cr.
are closely associated in V D
topography,function and Ca.
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 Gonad Mesonephros
the other. (nephrogenic
Plate)
The UGR is longitudinal swelling in (genital ridge)
dorsolateral side of the abdomen
UGR--> formed mostly from
Mesonephric
--non-segmented intermediate mesoderm
Duct
Lateral UGR(nephrogenic plate)
Paramesonephric
forms urinary organs and internal duct
genital ducts.
Ventromedial UGR is genital ridge, The Urogenital ridge
forms gonads.
 SEX DETERMINATION

Red arrows shows gene regulation Mullerian/

Paramesonephric
duct

DAX Ovary Oestrogen Female genital

Wnt 4
ducts
Bipotential
Gonadal ridge gonad
SRY Testis Anti-Mullerian hormone
(Sertoli cells)
Primary sex determination at fertilisation Regression of Mullerian
(i) genetic sex: XY, XX duct
(ii).Y chromosomes encodes testis- Testosterone
determining (Leydig cells)
factorSRYgonadogenesis
secretion of foetal hormones by
interstitial cells[(Sertoli and •Differentiation of Wolffian duct into
Leydig(M),theca cells(F)] Male genital ducts
secondary sex(phenotypic sex) •Descent of testis
 Gonadogenesis(2). The bipotential gonad
 Gonadogenesis occurs at the genital ridge initiated by 2 simultaneous events:-
(i).Formation of gonadal cords
 Epithelium 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.

PGC arrive at the genital Mesonephric/Wolffian Arteriole Dorsal aorta

duct Glomerulus
ridge at 21 days cat and
proliferate.
Epithelial incorporate PGC,
forming gonadal cords.
Gonadal differentiation
begins.

Dorsal
Mesonephric Genital mesentery Migratiing
tubule ridge PGC
 PROSTATE NEOPLASIA

BENIGN PROSTATIC HYPERPLASIA

AND
PROSTATE CANCER
 PROSTATE ANATOMY
• fibromuscular tissue (30-50%)
• glandular epithelial cells (50-70%)
• peripheral zone (most cancers)
• central zone
• transition 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 prevalence
• Some genetic and racial susceptibility to symptom
severity (autosomal dominant)
• Diet high in saturated fats, zinc and low in fruits and
vegetables.
• Sedentary life style.
 BPH
Proposed Etiologies
• Reawakening of the urogenital sinus to
proliferate
• Change in hormonal milieu with alterations in
the testosterone/estrogen balance
• Induction of prostatic growth factors
• Increased stem cells/decreased stromal cell
death
 BPH
Pathophysiology
• Slow and insidious changes over time
• Complex interactions between prostatic
urethral resistance, intravesical pressure,
detrussor functionality, neurologic integrity,
and general physical health.
 Etiopatogenesis
• Teori dehidrotestosteron (DHT)
5α -reduktase
Testoteron ------------------------------ DHT
• Teori tidak seimbang antara estrogen vs
testosteron
• interaksi stroma vs epitelial (growth factor)
• berkurangnya apoptosis sel
• Teori stem sel
 Pathophysiology
• Initial hypertrophydetrussor
decompensationpoor tonediverticula
formationincreasing urine
volumehydronephrosisupper tract
dysfunction
PENIS KARSINOMA
 TESTICULAR TUMOUR
1% of all Malignant Tumour
Affects young adults - 20 to 40 yrs - when
Testosterone Fluctuations are maximum
90% 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 Tumours
Improved overall survival in last 15 to 20 years
due to -
• Better understanding of Natural History and
Pathogenesis of disease
• Reliable Tumour Markers
• Cis-platinum based chemotherapy
 CROSS SECTION OF TESTIS
Testis

Stroma Seminiferous Tubules

(200 to 350 tubules)

Interstitial Cells Supporting

Spermatogonia
Leydig or
(Androgen) Sertoli Cell
 EPIDEMIOLOGY
Incidence : 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
 CLASSIFICATION

I. Primary Neoplasma of Testis.

A. Germ Cell Tumour
B. Non-Germ Cell Tumour

II. Secondary Neoplasms.

III. Paratesticular Tumours.

 I. PRIMARY NEOPLASMS OF TESTIS

A. Germinal Neoplasms : (90 - 95 %)

1. Seminomas - 40%
(a) Classic Typical Seminoma
(b) Anaplastic Seminoma
(c) Spermatocytic Seminoma
2. Embryonal Carcinoma - 20 - 25%
3. Teratoma - 25 - 35%
(a) Mature
(b) Immature
4. Choriocarcinoma - 1%
5. Yolk Sac Tumour
 I. PRIMARY NEOPLASMS OF TESTIS

B. Nongerminal Neoplasms : ( 5 to 10% )

1. Specialized gonadal stromal tumor
(a) Leydig cell tumor
(b) Other gonadal stromal tumor
2. Gonadoblastoma
3. Miscellaneous Neoplasms
(a) Adenocarcinoma of the rete testis
(b) Mesenchymal neoplasms
(c) Carcinoid
(d) Adrenal rest “tumor”
 II. SECONDARY NEOPLASMS OF TESTIS

A. Reticuloendothelial Neoplasms
B. Metastases

III. PARATESTICULAR NEOPLASMS

A. Adenomatoid
B. Cystadenoma of Epididymis
C. Mesenchymal Neoplasms
D. Mesothelioma
E. Metastases
AETIOLOGY OF TESTICULAR TUMOUR

1. Cryptorchidism

2. Carcinoma in situ

3. Trauma

4. Atrophy
 CRYPTORCHIDISM & TESTICULAR TUMOUR

Risk of Carcinoma developing

in undescended testis is

14 to 48 times the normal

expected incidence
 CRYPTORCHIDISM & TESTICULAR TUMOUR

The cause for malignancy are as follows:

• Abnormal Germ Cell Morphology
• Elevated temperature in abdomen &
Inguinal region as opposed to scrotum
• Endocrinal disturbances
• Gonadal dysgenesis
 Requirements for staging

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, AFP
 Investigation
1. Ultrasound - Hypoechoic area
2. Chest X-Ray - PA and lateral views
3. CT Scan
4. Tumour Markers
- AFP
-  HCG
- LDH
- PLAP
 CLINICAL FEATURES

• Painless Swelling of One Gonad

• Dull Ache or Heaviness in Lower Abdomen
• 10% - Acute Scrotal Pain
• 10% - Present with Metatstasis
- Neck Mass / Cough / Anorexia / Vomiting / Back
Ache/ Lower limb swelling
• 5% - Gynecomastia
• Rarely - Infertility
 Tumour Markers
TWO MAIN CLASSES
• Onco-fetal Substances : AFP & HCG
• Cellular Enzymes : LDH & PLAP
( AFP - Trophoblastic Cells
HCG - Syncytiotrophoblastic Cells )
 AFP –( Alfafetoprotein )
NORMAL VALUE: Below 16 ngm / ml
HALF LIFE OF AFP – 5 and 7 days

Raised AFP :
• Pure embryonal carcinoma
• Teratocarcinoma
• Yolk sac Tumour
• Combined Tumour

REMEMBER: AFP Not raised is Pure Choriocarcinoma or Pure Seminoma

 HCG – ( 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 Tumour
7% - Seminomas
 ROLE OF TUMOUR MARKERS
• Helps in Diagnosis - 80 to 85% of Testicular Tumours have
Positive Markers
• Most of Non-Seminomas have raised markers
• Only 10 to 15% Non-Seminomas have normal marker level
• After Orchidectomy if Markers Elevated means Residual
Disease or Stage II or III Disease
• Elevation 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 Burden
• Markers indicate Histology of Tumour:
If AFP elevated in Seminoma - Means Tumour has Non-
Seminomatous elements
• Negative Tumour Markers becoming positive on follow up
usually indicates -
Recurrence of Tumour
• Markers 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 Surgery
• Advanced Disease or Metastasis - Responds well to
Chemotherapy
 CONCLUSION

• Improved Overall Survival of Testicular

Tumour due to Better Understanding of the
Disease, Tumour Markers and Cis-platinum
based Chemotherapy
• Current Emphasis is on Diminishing overall
Morbidity of Various Treatment Modalities
SEMINOMA