Bellinii ducts.
Incidence:
7%000 population
2 % in adult cancer
M / F ratio = 2/1
Etiology:
smoking, Cd, Pb, asbestos, heritable changes in gene
chromosome 3p,
somatic mutations of the VHL gene, chromosomes
1 and 8 T. Bellini, chromosomes 7, 17 and Y in T.
tubulo-papillary.
V.H.L. gene
hypermetilation
DNA genetic mutation
Cytologic forms :
clear cell carcinoma (60-85%), round and
polygonal with glycogen and lipid-rich content;
granular cell carcinoma (acidophilus) (7-14%)
rich in mitochondria and organelles;
spindle cell carcinoma (4-10%) considered highly
malignant (basophils);
cancer cell oncocitice(2-5%);
Bellini duct cancers(1-2%).
Nuclear grades (FUHRMAN)
• regular outline of the nucleus
Gr 1. • nucleoli absent or minimal
• normal cells
• irregular nucleoli
Gr 3. • visible nucleoli x 100
• Multilobat nucleus
Gr 4. • visible nucleoli xx
• cellular monstrosities xx
Macroscopic
Tumor volume:
variable;
The existence of
pseudo-capsule at the
periphery of the
tumor;
Hyper-or hypo-
vascularised.
Tumor extension
a) Local: large tumors that may invade
surrounding structures and organs (T);
b) Lymphatic: regional lymph nodes (N);
c) Venous:
Renal vein thrombus and / or V.C.I.:
floating or adherent thrombus;
Metastases.
Circumstances of diagnosis
Urological signs :
1) Hematuria
2) Lumbar pain
3) Lumbar swelling
4) Varicoceles (newly emerging on the
right)
Non-urological signs :
1) Alteration of the general state
2) Prolonged febrile syndrome
3) Revealing metastases
4) High blood pressure
5) Occasional findings
6) Paraneoplastic syndromes
Paraneoplastic syndromes
4. Rare:
1. Hematologic: 2. Non metastatic
- Neuropathies,
hepatomegaly
- Polyglobulia, characterized by: - Neuro-myopathy,
- Thrombocytosis, - Retention B.S.P., - Paraproteinemias
- - ↓ prothrombin 3. Hypercalcemia IgM
Hypereosinophilia, index, - Liver or hepato-
- Lymphocyte type splenic
- ↑ alkaline
leucemoide amyloidosis,
phosphatase and
reactions. indirect bilirubin - Cushing sdr.
reversible
Biologic
Other exams:
examinations:
• ESR, • Ultrasound;
• CBC, • Urography (I.V.U);
• Serum calcium, • C.T. or R.M.N.;
• Hepatic balance • Renal arteriography;
• Phlebography;
• Cytology: free flow,
percutaneous
puncture;
• Lung Rx;
• Bone radiographs;
• Lymphoscintigraphy.
After
injection of
the contrast
medium:
image
suggestive
of renal cell
carcinoma
Hydronephrosis,
Splenomegaly.
Differential diagnosis(2)
Prolonged febrile syndrome:
septicemia,
infectious endocarditis,
brucellosis.
chiste renale,
xanto-granulomatous pyelonephritis.
Classification of ROBSON
• tumor limited to the renal parenchyma in
Std I: compliance with the capsule.
Metastasis
Regional lymph nodes
Primary Tumor • T0 – no evidence of PT. lymph nodes distant
• T1a – tu. <4 cm, limited to cannot be metastasis
the kidney. assessed • M1 Distant
• T1b – tu. between 4 - 7 cm, • N0 No metastasis
limited to the kidney. regional
• T2a – tu. between 7 - 10 lymph node
cm, limited to the kidney. metastasis
• T2b – tu. >10 cm, limited • N1
to the kidney.
Metastasis in
• T3a - extension in renal
vein, adrenal gland, a single
perirenal fat, but not regional
beyond of Gerota’s fascia. lymph node
• T3b – extends in VCI • N2
below diaphragm. Metastasis in
• T3c – extends in VCI more than
above diaphragm. one regional
• T4 – invades beyond of lymph node
Gerota’s fascia.
V: - Venous invasion
(after surgery)
V1: - renal vein invasion;
V2: - invasion of the inferior vena cava
below the hepatic veins;
V3: - invasion of the inferior vena cava
above the hepatic veins.
T.N.M.
Robson classification
(UICC-1992)
Std I: limited at the kidney T 1-2 N0 M0
Std II: limited at the renal loge T3a N0 M0
Std IIIa: invades renal vein (vena cava) T3a N0 M0
metastases (M)
febrile form
General:
developed from metanefrogen tissue (Blastem
metanefrogen);
1-5 years;
Macroscopic:
voluminous renal
tumor,
encapsulated, of
variable consistency
(firm → friable)
Microscopic:
Originof the blastema metanefrogen
consisting of three cell types:
blastematoasis (very young faults);
epithelial (rudimentary tubules and
glomeruli);
stromal (mezenchimatoase
undifferentiated or sometimes muscle, fat,
bone or cartilage).
Clinical:
Large renal tumor(83%)
Haematuria (21%)
Pain (due to intratumoral hemorrhage)
pseudo-digestive forms;
pseudo-appendiceal forms.
High blood pressure(63%);
Declive edema + / - ascites;
Associated anomalies: urogenital, ocular or
growth disorders.
Urogenital malformations:
urinary: duble or pyelo-uretheral bifidity,
horseshoe kidney
genital: hypospadias, testicular ectopia;
nephrology: nephrotic syndrome.
Eye malformations:
aniridia, microphthalmia, cataract;
Growth disturbance:
hemi-hypertrophy corporeal, visceromegalia.
Further examination
Ultrasound
R.S.
U.I.V.
T.C.: - with and without contrast agent.
Other examinations:
Pulmonary radiography and tomography,
Bone scintigraphy.
Wilms tumor classification
• unbroken preoperative tumor confined to the
Std I: kidney capsule without invading their
Non-epithelial:
angiomyolipoma;
veryrare: fibroids, leiomyoma, lipoma,
lymphangioma, hemangioma, mesoblastic
nephroma;
Fluid cysts
Right renal cyst - Bosniak class II
Left renal cyst - Bosniak class III
Left renal cyst - Bosniak Class IV