Causes, pathogenesis, Morphology, stages and prognosis

1. Renal Cell Carcinoma
Lecturer 53
Tumors of the Kidney
Adenocarcinoma of the Kidney
Hypernephroma

2. HYPERNEPHROMA- RCC
• Because of their gross yellow color and the
resemblance of the tumor cells to clear cells
of the adrenal cortex, they were at one time
called Hypernephroma.

3. Renal Adenocarcinoma
• It is now clear that all these tumors arise
from tubular epithelium
and are therefore
RENAL
ADENOCARCINOMAS.

4. Epidemiology
• Male predominance (1.6:1.0 M:F)
• Highest incidence between age 60-80
-Median age of diagnosis is 66 years
-Median age of death 70 years
• Highest incidence in Scandinavia and North
America, lowest in Africa

5. Risk Factors

6. Causes/ Risk Factors
• There is also an increased incidence in
patients with chronic renal failure
and acquired cystic disease and in
tuberous sclerosis.

7. RCC
• Most renal cancer is
sporadic, but unusual forms of
autosomal dominant familial
cancers occur, usually in
youngerindividuals.

8. Classification of RCC
The major types of RCC are as follows:
• 1. Clear cell carcinoma -70-80%, 95%
Sporadic
• 2. Papillary carcinoma- 10-15%
• 3. Chromophobe renal carcinoma- 5%
• 4. Collecting duct (Bellini duct) carcinoma-
1% 0r less

11. Pathogenesis of VHL
• Von Hippel-Lindau protein, product of VHL
gene, is a tumor suppressor
• VHL inhibits hypoxia-inducible genes involved
in angiogenesis such as VEGF, TGF-a, GLUT-1
• VHL destabilizes and promotes ubiquination of
HIF-a (hypoxia-inducible factor)
• Loss of VHL results in tumor angiogenesis,
tumor-cell proliferation, epithelial cell
proliferation
Glucose transporter 1
Ubiquination: a small polypeptide, found in most eukaryotic cells, that combines with other
proteins to make them susceptible to degradation

12. Morphology
•Renal cell carcinomas may
arise in any portion of the
kidney, but more
commonly affects the
poles.

14. RCC Morphology
• One of the striking
characteristics of renal cell
carcinoma is its tendency to
invade the renal vein and grow
as a solid column of cells within
this vessel.

15. Clear cell Carcinomas

20. Morphology- Papillary Carcinomas
• Papillary carcinomas are the most common
type of renal cancer in patients who develop
dialysis-associated cystic disease.

21. Microscopy- Papillary Carcinoma
• Papillary carcinoma is composed of cuboidal
or low columnar cells arranged in papillary
formations.
• Interstitial foam cells are common in the
papillary cores.
• Psammoma bodies may be present.
• The stroma is usually scanty but highly
vascularized.

22. Chromophobe renal carcinoma
• Chromophobe renal carcinoma is made up of
pale eosinophilic cells, often with a
perinuclear halo, arranged in
solid sheets with a concentration of
the largest cells around
blood vessels.

23. Collecting duct carcinoma
• Collecting duct carcinoma is a rare variant
showing irregular channels lined by highly
atypical epithelium with a hobnail pattern.

24. RCC
•Sarcomatoid changes
arise infrequently in all types of renal cell
carcinoma and are a decidedly
ominous feature.

25. Renal cell carcinoma. Typical cross-section of yellowish, spherical neoplasm
in one pole of the kidney. Note the tumor in the dilated thrombosed renal vein.

27. Paraneoplastic Syndromes
1. Polycythemia,
2. Hypercalcemia,
3. Hypertension,
4. Hepatic dysfunction,
5. Feminization or masculinization,
6. Cushing syndrome,
7. Eosinophilia,
8. Leukemoid reactions, and
9. Amyloidosis.

28. RCC

29. Clinical Presentation
• Most asymptomatic
• Hematuria present 40% of patients
• Classic triad: flank pain, hematuria, palpable
abdominal mass occur in 9% of patients
• 45% present with localized disease,
• 25% with locally advanced disease,
• 30% with metastatic disease

32. Treatment
•Nephrectomyhas been the
treatment of choice, but
•Partial nephrectomy to
preserve renal function is being done with
increasing frequency and similar outcome.