world cancer report - iarc

Stage Clear cell carcinoma Papillary carcinoma
Adenoma Loss of 3p Loss of Y chromosome
Partial trisomy of 5q Trisomy of 7, 17
Loss of Y chromosome Gain of 3p
Gain of 7, 12, 16, 17, 20
Carcinoma p53 mutations Loss of 6q, 9, 11, 14q, 17/17p, 21
Loss of 8, 9, 13, 14, 6q, 10q,
18q, 11, 17/17p Gain of 8, 20
Gain of 12, 20 Loss of MET function
Loss of VHL function
Metastatic tumours Excess of minichromosomes,
comprising 7q31 containing
the MET oncogene
Table 5.15 Genetic alterations in renal cell carcinoma.
ciated with structural alterations of the
short arm of chromosome 3 and with VHL
gene mutations [1,11].
Wilms tumour of the kidney occurs in
both sporadic and familial forms. It has a
specific syndrome associated with abnormalities
including aniridia (absence of the
iris), hemihypertrophy (overgrowth of one
half of the body or a body part), and cryptorchidism
(failure of the testes to
descend into the scrotum). A number of
loci involved in the development of Wilms
tumour have been characterized, key
amongst these being WT1, a tumour suppressor
gene located on chromosome
11p [12].
Fig. 5.148 Five-year relative survival after diagnosis
of kidney cancer.
Management
Radical nephrectomy (removal of the
kidney, perinephric fat, adjacent lymph
nodes and often the adrenal gland) is
currently the main therapy for renal cell
carcinoma. This procedure has been
shown to produce better survival rates
than simple nephrectomy (kidney
removal only), since involvement of
regional lymphatics and periaortic lymph
nodes has been noted in almost 25% of
patients [1]. Treatment for transitional
cell carcinoma is radical nephroureterectomy,
although more conservative
therapy may be appropriate for
smaller low-grade tumours. In patients
possessing a single kidney, or in the
case of bilateral simultaneous tumour,
either partial nephrectomy or radical
nephrectomy with dialysis and possible
later transplantation is indicated [1].
However, immunosuppression associated
with transplantation raises the risk of
potential tumour recurrence (Immunosuppression,
p68).
Accurate staging depends on histological
evaluation of the resected tumour.
Up to 30% of patients present with
metastases at diagnosis or relapse following
surgery. Metastatic kidney cancer
is extremely resistant to systemic
therapy [13]. A potential reason for this
is the high level of expression of the
multi-drug resistance gene MDR1 which
encodes P-glycoprotein (Box: Resistance
to cancer chemotherapy, p285) in both
normal proximal tubules and in tumour
Fig. 5.146 Surgical specimen of a bisected kidney
showing a large renal cell carcinoma. Much of the
kidney has been replaced by tumour tissue.
Fig. 5.147 Clear cell carcinoma of the kidney
showing a monomorphic proliferation of distinctive
tumour cells, with an abundant clear, lipidcontaining
cytoplasm, arranged in a trabecular
pattern.
tissue [14]. Most chemotherapeutic and
hormonal agents appear to show little
efficacy, although there is some controversy
over the efficacy of vinblastine and 5fluorouracil
as single agents or in combination
therapy [13,8]. In contrast, in the
treatment of transitional cell carcinoma,
cisplatin combination therapy seems to
be effective.
For the systemic treatment of metastatic
kidney cancer, interferon-α and interleukin-2
have been shown to elicit a
modest response rate of 10-15% [8],
allowing complete response in some
patients and an increased survival benefit
in others. Although overall survival
rates are poor (Fig. 5.148), the five-year
survival for patients with early stage
tumours is greater than 80% [8]. The
indication of renal vein/inferior vena
Kidney cancer
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