world cancer report - iarc

Fig. 5.144 A patient receiving kidney haemodialysis:
long-term dialysis predisposes to acquired
cystic disease of the kidney which may increase
the risk of subsequent cancer.
T
VC
Fig. 5.145 Magnetic resonance image of a renal
cell carcinoma (T), with a tumour thrombus in the
inferior vena cava (VC).
Detection
Kidney cancer commonly causes no
obvious symptoms in its early stages.
Subsequently, symptoms include haematuria,
loin pain and a palpable kidney
mass [8] and these usually indicate
patients with advanced disease. As a
consequence of increasing use of renal
imaging techniques, increasing numbers
of asymptomatic, incidental tumours are
being detected [5]. Diagnosis of renal
cell carcinoma may be preceded by
paraneoplastic syndromes, the systemic
and humoral manifestations of the disease,
which result from the overproduction
of normal kidney proteins or hormones
(e.g. renin, erythropoetin,
prostaglandins) or inappropriate expression
of non-kidney factors (e.g. parathyroid
hormone). Symptoms may include
hypertension, fever, anaemia, erythrocytosis
(elevated number of red blood
cells), abnormal liver function and hypercalcaemia
(abnormally high calcium levels)
[2, 8].
262 Human cancers by organ site
The presence of a tumour may be initially
defined by intravenous urogram. Computed
tomography (CT) is the imaging
procedure of choice for diagnosis and
staging [1]; scanning of the abdomen
and pelvis confirms tumour extent,
lymph node status and contralateral kidney
functionality. Selective renal arteriography
via percutaneous femoral artery
catheterization may be used for diagnosis
and staging [1]. Less invasive than
arteriography is magnetic resonance
imaging (MRI), which can also be used to
assess thrombus of renal vein or vena
cava involvement (Fig. 5.145). Chest
radiographs (commonly with CT) and
technetium-99m radiopharmaceutical
bone scans are employed to determine
whether lung or skeletal metastases are
present.
Pathology and genetics
Renal cell carcinoma (Figs. 5.146, 5.147)
is commonly represented by adenomas,
although there is some controversy over
the difference between renal cortical adenoma
and renal cell adenocarcinoma [1].
In terms of renal cell carcinoma histology,
grade I cells have a lipid-rich cytoplasm
and a small peripheral nucleus. As grade
advances from I to IV, the nuclear pleomorphism
increases and the lipid-rich
cytoplasm reduces. The tumour is initially
capsulated (in 50-60% of diagnosed
cases), tends to spread to lymph nodes
(10% of cases diagnosed) or may metastasize
to the lungs, bone, brain and liver
(20-30% of cases). There is a tendency for
the tumour to spread within the renal vein
and into the inferior vena cava, extending
in extreme cases into the right atrium [8].
Transitional cell carcinoma accounts for
5-8% of kidney tumours [8] and is derived
from the renal pelvis transitional cell
epithelium, which is identical to that of
the bladder and ureter; 50% of patients
with renal transitional cell carcinoma also
develop the same tumour type of the
bladder.
Cytogenetics and molecular biology have
allowed significant advances to be made
in the differentiation and staging of kidney
cancer tumours, which may be histologically
complex and heterogeneous [9].
Cytogenetics have shown, for example,
that the two main types of renal cell carcinomas,
clear cell (non-papillary) carcinoma
and papillary carcinoma, are genetically
distinct (Table 5.15), although there
can often be difficulties in distinguishing
them histologically. Corresponding
changes in transitional cell carcinoma
have been less well-defined. The papillary
form has a better prognosis than the nonpapillary
[9]. Mitochondrial DNA changes
have been observed in early-stage oncocytic
and chromophobe tumours [10], but
are not yet used clinically.
Von Hippel-Lindau disease is characterized
by the development of multiple
tumours, including bilateral renal cell carcinoma,
pheochromocytomas, hemangioblastomas
of the central nervous system,
retinal angiomas and pancreatic
cysts [1,11]. Von Hippel-Lindau patients
have a >70% lifetime risk for renal cell
carcinoma and it is the cause of death in
15-50% of cases. Such patients thus
require regular screening; currently some
30-50% of patients with von Hippel-
Lindau disease who are identified with
renal cell carcinoma as a result of symptoms
have metastases on presentation,
and hence respond poorly to treatment.
Most families with von Hippel-Lindau disease
(80%) have mutations in the VHL
gene, a probable tumour suppressor
gene. Sporadic forms of renal cell carcinoma,
as well as familial forms, are asso-
Fig. 5.143 Mortality from kidney cancer in variouscountries.