RENAL TUMORS

Tumours of the kidney

 Benign tumours clinically
unimportant
 almost always small, so just incidental
findings - e.g. cortical adenoma found in
20% autopsies
 Metastatic tumours are uncommon,
despite massive renal blood flow
 Malignant tumours of the
kidney
The only important ones are -
 nephroblastoma (Wilms’ tumour)

 renal cell carcinoma

 transitional cell carcinoma of renal

pelvis (essentially part of urinary

tract)
 Nephroblastoma
(Wilms’ tumour)
 One of commonest intra-abdominal
tumours < 10 yrs age.
 Occasionally bilateral
 Mostly 1-5 years (can even be
congenital)
 Highly malignant tumour of mesoderm
(renal blastema) – often already spread
to lungs at time of diagnosis
 Nephroblastoma
 Usually quite big tumors
 They tend to be encapsulated and
vascularized and do not cross the
midline of the abdomen.
 Pathology
 A triphasic nephroblastoma comprises three
elements:
 blastema
 mesenchyme
 epithelium
 Contains tubules and glomeruli surrounded by a
spindled cell stroma. The stroma may include
striated muscle, cartilage, bone, fat tissue, fibrous
tissue. The mesenchymal component may include
cells showing rhabdomyoid differentiation. The
rhabdomyoid component may itself show features
of malignancy (rhabdomyosarcomatous Wilms).
Nephroblastoma
 PRESENTATION
Typical symptoms are:
 An abnormally large abdomen

 Abdominal pain

 Fever

 Nausea and vomiting

 Blood in the urine (in about 20% of

cases)
 High blood pressure in some cases
Wilms' tumors may be separated into
2 prognostic groups based on
pathologic characteristics:
 Favorable - Contains well developed

components
 Anaplastic - Contains diffuse

anaplasia (poorly developed cells)

Excellent results can be achieved if it
can be treated aggressively
 combination of radiotherapy,
nephrectomy and chemotherapy is
used.
 5 yr survival is about 90%.
 Renal cell carcinoma
(adenocarcinoma of the
kidney)
 commonest (~90%) renal malignancy
in adults.
 Age: 60 – 70 yrs and male: female 2:1
 usually large bulging tumour at renal
pole (upper > lower)
 yellowish cut surface, often with cysts
and haemorrhage, often has
apparently sharp margins, due to
pseudocapsule
 Renal Cell Cancer Risk
Factors
 Smoking: Cigarette smokers are twice as likely
as nonsmokers to develop kidney cancer.
 Long-term Dialysis: Being on dialysis for many
years is a risk factor for kidney cancer.
 Von Hippel-Lindau Syndrome: VHL is a rare
disease that runs in some families. An abnormal
VHL gene increases the risk of kidney cancer.
 Occupation: workers in the iron and steel
industry are at risk. Workers exposed to
asbestos or cadmium also may be at risk.
 Histology of renal cell CA
 derived from renal tubular cells
 80 % are “clear cell”
adenocarcinomas
 abundant clear or granular cytoplasm
- contain glycogen and fat
 other histological types (“papillary” and
“chromophobe”) have better prognoses
 classification of renal cell CA can now be
based on correlation of genetic and
histological changes
 Spread of renal cell
carcinoma
 local, lymphatic and blood
 may invade perinephric fat
 can invade pelvi-calyceal system
 lymphatic – first to para-aortic nodes
 often invades renal vein and IVC
 blood spread most often to lungs (50%),
bones (33%), adrenals and brain
Presentation of renal cell CA
usually late
 most often, haematuria

 abdominal mass +/- loin pain

one of the great “mimickers”

 Symptoms due to metastasis (classically

cannon ball metastases in lungs)

 fever of unknown origin/ night sweats

 weight loss, malaise

 paraneoplastic phenomena -

- secretion of erythropoietin, renin,

parathormone, ACTH etc
 TREATMENT
 Surgery is the only curative option
 Radical nephrectomy is the standard
treatment for renal cell carcinoma
 Nephron sparing partial nephrectomy
may be indicated in:
 Bilateral renal tumors
 Patients with Von Hippel Lindau syndrome

 Renal CA in a solitary kidney

 Metastatic Renal Cell
Carcinoma
 Interleukin 2, interferon gamma, anti
VEGF antibody therapy –
Bevacizumab, Tyrosine Kinase
inhibitors – Sunitinib,
sorefenib,Temsirolimus
 Prognosis of renal cell CA
5-yr survival rate overall ~ 50%
 T1a N0 M0 (< 4 cm tumor) 91-

100% 96-97% 89-100%

 T1b N0 M0 - 95-96%

 T2 N0 M0 - 88%

 T3 N0 M0 - 40-70%

 T4 N0 M0 - 0-28%

 N1 - 0-33%

 M1 - 0-20%
 Transitional tumours
 Age 50 – 70 yrs: men > women
 White > black > oriental populations
 Arise anywhere in urinary tract, but
bladder (especially bladder base) >
pelvis/calyces > ureters > urethra
 Often polypoidal / papillary/ fronded, - if
sessile (flat) - more likely to be muscle
invasive and of high grade
 May be preceded by dysplasia/CIS
Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 14 July 2008 01:09 PM)
© 2007 Elsevier
 Renal pelvis cancer
 Renal pelvis cancer is a malignant
tumour arising from the lining of the
renal pelvis of the kidney.
 Renal Pelvis Cancer
 Urine excreted by the kidney collects in the
renal pelvis. It is then passed down the
ureter to the bladder. The lining of the renal
pelvis comprises transitional epithelium
similar to that found in the bladder.
 Renal pelvis cancer is a transitional cell
carcinoma as apposed to an
adenocarcinoma that arises from the renal
cortex. Similar tumours arise in the ureter.
 INCIDENCE
 Transitional cell carcinomas account for 8%
of all kidney cancers.
 In 2 – 4% of patients the disease is bilateral.
 The incidence increases with age. Most
patients are over 65 years
 Renal pelvis cancer is more common in
males than females.
 The ratio is twice as many men as women.
 Predisposing factors
 In most cases the exact cause of renal pelvis cancer is
In most cases the exact cause of renal pelvis cancer is
unknown.
 Smoking is a causative agent in developing renal pelvis
cancer. The more cigarettes smoked in a day the higher the
risk. The risk is lowered if smoking is stopped.
 Some occupations have been linked to renal pelvis cancer.
These include
 aniline dye industry - Examples of these dyes include
benzidine, α and β naphthylamine.
 Hairdressers
 Mechanics
 Printing workers
 Cable manufacturing
 Painters and decorators
 Tyre manufacture
 Patients with a pre existing carcinoma of the bladder are at
risk of developing renal pelvis cancer.
 Phenacetin abuse has been associated with these cancers.
 Renal papillary necrosis predisposes to renal pelvis cancer.
 Types

 Transitional cell carcinomas.

 A small percentage will be squamous
cell carcinomas due to squamous
metaplasia in the lining of the renal
pelvis.
 Presentation
 One of the main presenting
symptoms is haematuria. Up to 50%
of these tumours present with this
symptom.
 The blood is usually throughout the
stream as the blood has time to mix
thoroughly with the urine. It is often
painless but occasionally the patient
will present with pain if there are
clots passing down the ureter.
Sometimes the blood is microscopic
in which case the patient will not
physically see the blood.
 PRESENTATION
 Some lesions present with abdominal or
loin pain.
 Ureteric tumours may present with pain
due to obstruction of the ureter.
 It is not uncommon for kidney tumours to
be without symptoms. In these instances
the tumour presents incidentally on a scan
such as an ultrasound scan or a CT scan.
 Other symptoms include tiredness, loss of
appetite, loss of weight and a raised
temperature (commonly referred to as a
PUO – pyrexia of unknown origin).
 Investigations
 Urine tests: It will detect the presence of
blood and white blood cells in the urine.
 Urine cytology is a specific test to detect
the presence of shed malignant cells in the
urine. A cytologist will detect these cells.
 Blood tests A full blood count will detect
anaemia.
 Ultrasound scans will pick up renal pelvis
tumours or hydronephrosis.
INTRAVENOUS UROGRAPHY
 Filling defects ( areas with no
contrast medium ) maybe seen on
the kidney views.
 The calyces may be distorted or
destroyed.
 Hydronephrosis may be detected.
 A normal IVU does not exclude the
presence of a renal pelvis tumour.
 CT SCAN
 Both contrast and non contrast
studies are performed.
 The size and the extent of the tumour
can be assessed thus staging it.
 Any involved lymph nodes will be
detected.
 Any mets can be detected
 Cystoscopy: If the patient presented
with haematuria then this
investigation is performed in order to
exclude any bladder pathology.
 A retrograde ureterogram is an xray
examination of the ureter and renal
pelvis performed by passing xray
contrast medium up the ureter. This
is performed at the time of
cystoscopy. It will identify filling
defects in the ureter and renal pelvis.
 Ureteroscopy: Ureteroscopy is an
endoscopic examination of the ureter
and renal pelvis. Both rigid and flexible
scopes can be passed up the ureter.
Tumours are identified under direct
vision.
 Biopsy: If the diagnosis is still in doubt
after the above investigations then a
percutaneous biopsy or a ureteroscopic
biopsy may be indicated.
 MRI scan: This scan is not routinely
performed. This scan may be useful
when the diagnosis is uncertain.
 Treatment
 Surgery offers the best chance of curing the
condition.
 Nephroureterectomy is the surgical removal
of the kidney with the entire ureter. Because
of field changes in the renal pelvis and
ureter this is the standard treatment.
 It is commonly performed through 2
incisions. The lower suprapubic incision
facilitates the excision of the lower ureter
with a surrounding cuff of bladder whilst the
upper renal incision allows the removal of
the kidney.
Modified nephroureterectomy : In this procedure the
basic principle of removing the kidney and ureter
with a cuff of bladder remains. The lower end of
the ureter is excised endoscopically from within
the bladder. This obviates the need for 2 incisions.
Segmental Ureterectomy: If the tumour is confined to
a small segment of ureter then it may be possible
to excise the tumour locally and anastomosing the
ureter to restore ureteric continuity. This allows the
patient to keep his kidney.

Radiotherapy: It can palliate pain or bleeding in those

tumours that are not surgically removed. It may
have a role in patients who are unfit for any
surgical procedure.
 Chemotherapy
 Intra-pelvic chemotherapy has occasionally been used.
 The long term results of this treatment have not been well reported.
 Chemotherapeutic agents used include thiotepa, Mitomycin-C,
Adriamycin (doxorubicin) and epi-rubicin.

 The long term results of systemic chemotherapy are also poorly

documented.
 Combination chemotherapy has produced the best results.
 Agents used include Methotrexate, Vinblastine, Adriamycin
(doxorubicin) and Cisplatinum - M-VAC regime.
 The M-VAC regime offers better response rates than Cisplatinum
alone. M-VAC has an overall response rate of 39% in Metastatic
disease.
 Follow up

 It is important that any patient diagnosed

with transitional cell carcinoma of the
renal pelvis must be followed up with
regular bladder cystoscopies.
 Up to 40% of patients will develop
recurrences in the bladder.
 If the tumour in the renal pelvis and ureter
is diffuse then the risk of bladder tumours
rises to 75%.
 Prognosis

 The tumour stage and grade have a

significant effect on survival.
 The grading of the tumour affects the
prognosis. Grades 1 and 2 have a
much better outlook than grade 3.
 The overall 5 year survival rate is
72% for all tumours. The non bladder
recurrence rate is 39%.