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Renal Tumors

Renal adenocarcinoma (R.C.C.)


 Definition:
Primitive malignant tumor of the renal
parenchyma

 Proximal convoluted tubule,


 Cortical collecting ducts,

 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

Tumor suppressor protein,


functionally inactive

↑HIF (hypoxia inducible factor)


Bevacizumab
VGF PDGF
VEGFR PDGFR
Axitinib

Sunitinib angiogenesis ↑ Tumor progression


Sorafenib by proliferation
Pathological Anatomy
Microscopic: renal adenocarcinoma = 97%

 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

• discrete irregular nucleus


Gr 2. shape
• visible nucleoli x 400

• 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

Masă tumorală rinichi drept înaintea administrării


substanţei de contrast
RMN (T2): Renal adenocarcinoma with thrombus in V.C.I.
Renal angiography:
neovascularization
typical of renal
adenocarcinoma,
lower pole, right
kidney.
Differential diagnosis(1)
 Hematuria
 The role of ultrasound, the U.I.V. and
cystoscopy.

 Palpable lumbar mass:


 Polycystic kidney,
 Solitary renal cyst,

 Hydronephrosis,

 Splenomegaly.
Differential diagnosis(2)
 Prolonged febrile syndrome:
 septicemia,
 infectious endocarditis,

 brucellosis.

 After ultrasound and urography are taken


into account:
 kidney cancer,
 renal angiomyolipoma,

 chiste renale,

 xanto-granulomatous pyelonephritis.
Classification of ROBSON
• tumor limited to the renal parenchyma in
Std I: compliance with the capsule.

• tumor beyond the capsule but


Std II: remains limited to renal box.

• A = invasion of renal vein or


inferior vena cava;
Std • B = invasion of regional lymph
III: nodes;
• C= Venous and lymphatic invasion

Std • A = adrenal invasion or other


adjacent organs;
IV: • B = distant metastases.
Classification of
ROBSON
T.N.M. classification
• Tx –PT cannot be assessed. • NX Regional • M0 No

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

Std IIIb: invades regional lymph nodes T1-3 N1-3 M0

Std IIIc: venous and lymphatic invasion T3b N1-3 M0

Std IVa: infiltration of adjacent organs T4 N0-3 M0

Std IVb: distant metastases T1-4 N0-3 M1


Progress
• Death - neoplastic metastasis and
Untreated
cachexia
• Nephrectomy:
• Healing by radical surgery;
• Improvement, if after nephrectomy was
left neoplastic tissue;
Treated • Excision of metachronous metastases is
possible and useful;
• Spontaneous regression of metastases
is unlikely.
• = Adjuvant immunotherapy.
Prognosis
 Prognostic factors:
 tumor size(T)
 neoplasic adenopathy(N)

 metastases (M)

 febrile form

 pronounced weight loss

 altered liver function tests


Treatment
 Principal = surgical
“EXTRAFASCIAL NEPHRECTOMY
WITH PRIMITIV RENAL PEDICLE
LIGATION”
Limits of
radical
nephrectomy,
the left side
Adjuvant = immunotherapy:
 Specific:  Nonspecific:
 Monoclonal antibody  B.C.G.
(MAb) that acts on the  I.F.N.-α
tumor-associated antigen  I.F.N.-β
(TAA);
 I.F.N.-α+ I.L.-2
 adoptive cell therapy (re-
administration of IL-2  T.N.F.-α
prepared in vitro +
autologous immune cell
culture);
 I.L.-2 + L.A.K. or +
T.I.L.
Nephroblastoma (Wilms tumor)

 General:
 developed from metanefrogen tissue (Blastem
metanefrogen);
 1-5 years;

 ratio B / F = 1/1, often bilateral (10%);

 1-2 cases per 1 million population per year;

 second child tumor after neuroblastoma tumor.


Pathologic Anatomy:

 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

• tumor may invade the capsule exceeds hilum,


large vessels, excretory cavities, lymph nodes
Std II: and nearby organs but that complete excision
is possible macroscopic

Std • tumor with metastases limited to the abdomen,


III: the possibility of incomplete excision

Std IV: • marrow metastases

Std V: • Bilateral tumors


Differential diagnosis

Non-renal tumors: Renal disease:


- Neuroblastoma: developed - Hydronephrosis,
from the neural crest, - Polycystic kidney disease,
- Corticosuprarenaloame, - Chistadenoame or
- Retroperitoneal teratoma, multilocular cysts,
- Malignant lymphoma, - Renal adenocarcinoma,
- Tumors of the pancreas - Mesoblastic nephroma,
and digestive pathways nephroblastomatosis
Evolution:
- untreated → DEATH
- treated → can be favorably
Treatment
 Surgical:transperitoneal nephrectomy;
 Radiotherapy 20 Gray;
 Chemotherapy: Vincristine, Actinomycin-
D, Adriamycin.
Therapeutic indications
Unilateral non- R.T. with
Bilateral R.T.:
metastatic R.T.: metastasis:
• Neoadjuvant • Chemotherapy • Neoadjuvant
chemotherapy; (reduction in chemotherapy
• Nephrectomy; tumor volume); (triple
• Adjuvant • Surgery: combination);
chemotherapy; conservative / • Neoadjuvant
• Radiotherapy in radical; radiotherapy;
stage II and III • Chemotherapy • Transperitoneal
if lymph nodes and adjuvant nephrectomy
were affected radiotherapy.
Results
 Stage I-II N0 = 90-95%
 Stage II N+ = 70%
 Stage III = 70%
 Stage IV = 50%
Benign tumors
 Epithelial:
 papillaryadenoma; oncocitomul;
 metanephric adenoma;
 juxtaglomerular tumor cells;

 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

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