Eble JN, Sauter G., Epstein JI, Sesterhenn IA - iarc

and patients with pT 2 tumours have a
survival rate of 75% {1003,1834}.
Survival for patients with pT 3 and pT 4
tumours, tumours with positive nodal disease
and residual tumour after surgery is
poor {1995}. Other prognostic factors
include patient age, type of treatment,
and presence and severity of concurrent
urothelial neoplasia {163,2884}.
Squamous cell carcinoma
Squamous cell carcinoma is more common
in the renal pelvis than in the ureter,
although it is the next most common
tumour after urothelial carcinoma, it is
very rare in both locations. Pure squamous
cell carcinomas are usually high
grade and high stage tumours and frequently
invade the kidney. These
tumours may occur in the background of
nephrolithiasis with squamous metaplasia.
Survival for 5 years is rare {248}.
Adenocarcinoma
Pure adenocarcinomas of the renal
pelvis and ureters are rare and enteric,
mucinous or signet-ring cell phenotypes,
often occur concurrently. Glandular
(intestinal) metaplasia, nephrolithiasis
and repeated infections are predisposing
factors. Most adenocarcinomas are
high grade and are widely invasive at
presentation {590}.
Benign epithelial tumours
Urothelial papilloma and
inverted papilloma
Urothelial papilloma is usually a small,
delicate proliferation with a fibrovascular
core lined by normal urothelium. It is
extraordinarily rare and often found incidentally.
Inverted papilloma is also rare
being twice as common in the ureter as
in the renal pelvis. Most lesions are incidentally
discovered.
Villous adenoma and squamous
papilloma
These benign tumours are rare in the
upper urinary tract. The presence of a villous
adenoma histology in a limited biopsy
does not entirely exclude the possibility
of adenocarcinoma, and complete
excision is essential.
Non-epithelial tumours of renal
pelvis and ureter
Malignant tumours
The most frequent malignant stromal
tumour of the ureter is leiomyosarcoma.
Other malignant tumours reported are
rhabdomyosarcoma, osteosarcoma,
fibrosarcoma, angiosarcoma, malignant
schwannoma, and Ewing sarcoma {416,
506,657,746,1745,1925,2634}.
Benign tumours
Fibroepithelial polyps are exophytic intraluminal
masses of vascular connective
tissue and varying amounts of inflammatory
cells, covered by normal transitional
epithelium. These are most frequently
seen in the proximal ureter in young male
adults and, in contrast to urethral polyps,
children are rarely affected {2828}. Renal
pelvic and ureteric leiomyoma, neurofibroma,
fibrous histiocytoma, haemangioma,
and periureteric lipoma, including
hibernoma, have been reported {91,974,
1456,2449,2573,2712,2870}.
Miscellaneous tumours
Neuroendocrine tumours
Few cases of ureteric phaeochromocytoma
have been reported {128}. Pelvic
and ureteric carcinoid is similarly rare
{45,1217,2260} and must be differentiated
from metastatic disease {231}.
Carcinoids also occur in ureteroileal conduits
{1343}. Small cell carcinoma of the
renal pelvis is confined to elderly patients
{971,1347}. These aggressive tumours
usually contain foci of urothelial carcinoma
{971,1321,1326} and have a typical
neuroendocrine immunohistochemical
profile {971,1326,1347}.
Lymphoma
Renal pelvic and ureteric lymphomas are
usually associated with systemic disease
{200,331,2635}, while localized pelvic
plasmacytoma has been reported
{1165}.
Other
Rare cases of sarcomatoid carcinoma of
the pelvis and ureter can show either
homologous or heterologous stromal elements
{621,774,2727,2882}. The
tumours may be associated with urothelial
carcinoma in situ {2727,2882} and
have a poor prognosis {621,774,2882}.
Wilms tumour confined to the renal pelvis
or extending into the ureter {1114} and
cases of malignant melanoma and choriocarcinoma
of the renal pelvis have been
described {669,800,2680}.
Tumours of the renal pelvis and ureter 153