0022~5347 /83/i292-02?

5$0200G/0
THE JOURNAL OF UROLOGY
Copyright© 1883 by The -;Niliiarns & V/iildns Cc,

CARCINOMA OF THE URETER: NATURAL }VIANAGEiv1ENT

AND SURVIVAL
CARL MILLS AND K DARRACOT'l"' VAUGHAN, JR.
From the Department of Surgery, Division of Urology, Cornell University Medical College and The James Buchanan Brady Foundation,
New York Hospital, New York, New York

ABSTRACT
We reviewed retrospectively 53 new cases of primary ureteral carcinoma. Each patient was staged
pathologically and survival by operative procedure was compared. Depth of tumor invasion is the
most accurate indicator of over-all outcome. Patients with stage A tumors had an 80 per cent 5-year
survival rate, compared to only 33.3 per cent of the patients with stage C lesions. Patients also were
divided into 2 groups: group 1 patients had a mreteral lesion only, and group 2 patients had a u.reteral
tumor and a second primary u:rothelial lesion. No difference was found in 5-year survival rates for
patients in groups l (48.3 per cent) and 2 (47.7 per cent). Since prognosis for patients with low stage
tumors was not enhanced by an aggressive operation a more conservative approach is indicated.

Carcinoma of the ureter is a rare malignancy. 1 Batata and
associates reported on 2,500 urothelial tumors but found only
63 ureteral tumors.2 Other investigators also have supported
the rarity of this lesion. 3- 5 McCarron and Marshall recently
reviewed 1,431 cases of bladder carcinomas seen in a 40-year
period. During this same interval 69 patients with ureteral
tumors were treated. Twinem earlier reported on 16 cases of
ureteral carcinomas managed at our institution from 1932 to
1957. 7 We herein report on the remaining patients treated since
that time.
MATERIALS AND METHODS
A retrospective review of patients with primary ureteral
neoplasms managed from January 1957 to December 1978 was
completed. We found 53 new cases since those presented by
Twinem. 7 Each patient was staged pathologically by a modifi-
cation of Jewett's bladder staging system: 0-papilloma, A-
submucosal infiltration, B-muscular invasion, C-periureteral
fat invasion and D-tumor outside the ureter, regional lyrnph
nodes and distant metastases.
Patients also were divided into 2 groups: group 1 patients had
a ureteral tumor only and group 2 patients had either a previous
urothelial tumor, an unsuspected second lesion or later suffered
another urothelial carcinoma,
The presenting symptoms of patients with low stage lesions
were compared to those with high tumors and the treat-
ment for each stage vvas analyzed 1).
included l"C.niYC01,TN>1Cc<>Y•C,r,fm=-' with bladder ~,s,"oQ1CVU (1 or
2-stage), "t1,1ucucm resection, distal ureterectomy or local ex-
CIS10R
had stage D disease. The 3 patients with abdominal masses and
the 2 identified at autopsy did not have IVPs.
Of the 5 patients with stage O lesions 4 had been at risk for
~5 years. These patients all survived without tumor >5 years
and there is no difference in survival by group (table 4),
Of the 20 patients with stage A tumor 15 had been at risk for
>5 years. Of these 15 patients 12 (80 per cent) were free of
tumor at 5 years. Two died of cardiovascular
while l died of metastatic urothelial tumor. This patient also
had a history of low stage bladder carcinoma. Comparison of
patients in groups l and 2 with stage A carcinonia reveals
similar survival data. Each group has an over-all 5-year outcome
of 80 pe:r cent (table 3). In addition, 1 death was secondary
to metastatic disease.
Of the 7 patients with stage B ureteral carcinoma 6 had been
at risk for >5 years and 3 (50 per cent) were free of tumor. Two
patients died of a myocardial infarction and l died postopera-
tively. No patient died of the tumor. There were 3 patients with
stage B lesions in each gToup and 2 natlejnts (66.7 per cent) in
group 1, while l patient (33.3 per cent) in group 2 lived ~5
years.
Of the 6 patients with stage C malignancy 2 (33.3 per
cent) were alive >5 years. Both patients had known residual
tumor. All deaths have been secondary to metastatic disease.
The 2 ""-"'"°'"M alive at 5 years represent l from group l and 1
from
lS natmnts with stage D tumor lived >5
and for
imcidenta!h at autopsy. The

RESULTS
Of the 53 cases studied 34 ·were in group 1 and 19 were in
group 2 (table 2). The most common presenting symptom was
gross hematuria, which was found in 28 patients (table Most
of these patients (17 of 28) had low stage lesions (0 or A). The
next most common complaint was flank pain, which was noted
in 9 patients. Other initial complaints or findings were varied,
including microhematuria in 5, infection in 2, calculous disease
in 2 and metastatic abdominal disease in 3. In these last patients
autopsy confirmed the ureter as the primary source.
An excretory urogram (IVP) was done in 48 cases. A surpris-
ing finding was a high incidence of nonvisualization (22 of 48
cases). As would be expected nonvisualization often was accom-
panied by a high stage lesion (14 of 22 cases) and 10 patients
Accepted for publication March 12, 1982.
275
DISCUSSION
We reviewed and staged pathologically 53 new cases of ure-
teral tumor. Of the 53 patients 46 were at risk for s:5 years and
survival by stage is reported.
Stage O patients have an excellent prognosis and, hence, the
effect of the various surgical procedures for low stage lesions
did not appear to influence survival. All of the patients, regard-
less of treatment, are alive without tumor.
Of 15 patients with stage A disease 12 (80 per cent) lived ~5
years. Comparison of treatment indicates that each operation
was effective in this group of patients, although the number of
patients is small. Of the 12 patients treated with nephroureter-
ectomy and bladder cuff excision (1 or 2 stage) 10 lived 5 years.
Less extensive procedures were done in 3 patients and l of
these died postoperatively, while the remaining 2 are free of
276 MILLS AND VAUGHAN

TABLE 1. Stage
0 A B C D
Total Survival Total Survival Total Total Total
Pts.* %
Pts.' % Pts.'
Survival %
Pts.'
Survival % Pts.* Survival %

Local excision 1 100

Nephrectomy, partial ureterec- It 100 0 0
tomy
Nephroureterectomy with blad- 2 2 100 12 10 (5t) 80 4 2 50 5 2 40 7 14
der cuff excision
Segmental resection 2 50
Distal ureterectomy 1 100 2 50 0 0
Exploration, biopsy tumor 4 0 0
No operation -1.
Totals 4 4 100 15 12t 80 6 3§ 50 6 211 33.3 15 BIi
* Total at risk for 5 years.
t Distal ureterectomy as second operation.
t Died of transitional cell carcinoma.
§ No death of metastatic transitional cell carcinoma.
II All deaths of metastatic transitional cell carcinoma.

TABLE 2. Breakdown according to group and stage of 53 patients
with carcinoma of the ureter
Stage Group 1
0 3
A 12
B 4
C 4
D 11
Totals 34
TABLE 3. Presenting symptoms
Gross hematuria
Microhematuria
Flank pain
History of infection
History of stones
Constitutional symptoms
Abdominal, pelvic mass
Incidental autopsy
Total
TABLE 4. Survival for 5 years of patients according to group and
stage of disease
Stage
Group 1
No. (%)
0 2/2 (100)
A 8/10 (80)
B 2/3 (66.6)
C 1/3 (33.3)
D 1/11 (9)
tumor. These results show that patients with low stage lesions
have a good prognosis, and that the surgical approach appar-
ently does not influence the final outcome. Comparison of
patients in groups 1 and 2 with stage A tumors reveals similar
survival data, both groups having an 80 per cent survival rate
at 5 years. History of a urothelial carcinoma, the concomitant
finding of another urothelial lesion or the later development of
a second tumor did not alter the natural course of the original
lesion.
Analysis of patients with stage B lesions reveals an over-all
5-year survival rate of 50 per cent. Recently, an improved 5-
year survival rate for patients with this stage of disease was
reported. 8 Closer review of our data shows that none of our
patients died of the malignancy and that the surgical procedure
did not alter the final outcome. The number of patients with
this stage of disease is small but suggests that the natural
history for patients with stage B ureteral tumor is more favor-
able than reported previously. 2
Patients with deeply invasive ureteral neoplasms have a
worse prognosis than those with low stage lesions. Beck and
associates noted that the survival rate of patients depended on
the depth of tumor invasion. 4 Patients with highly invasive
tumors had a worse prognosis than those with superficial low
Group 2 stage lesions. Our data support this finding.
2 Patients with stage C disease had a poor outcome, even with
8 aggressive surgical therapy. Only 33.3 per cent of these patients
3 (2) lived 5 years and both patients have known recurrent
2 tumors. One patient had undergone distal ureterectomy and
4
lived only a few months. The remaining patients had a neph-
19
roureterectomy and bladder cuff excision, and the 2 survivors
are in this group. Although the results are disappointing an
aggressive surgical approach offers some hope. The number of
No. Cases patients is small so that no significant comparison by procedure
28 is possible.
5 The insidious onset of stage D disease and dismal prognosis
9 for patients are demonstrated. Most patients with gross hema-
2
2 turia (17 of 28 cases) had a low stage tumor that was diagnosed
2 easily. In contrast, patients with high stage carcinomas pre-
3 sented with mild flank pain (4 of 15), abdominal and pelvic
2 masses (3 of 15), and constitutional symptoms, such as weight
53 loss, fatigue and anorexia (2 of 15). Two patients were found at
autopsy for cardiovascular deaths to have metastatic ureteral
tumor. Only l patient lived 5 years and this patient has known
recurrent tumor.
Group 2 The multiplicity of urothelial tumor is well recognized. 9 Our
No. 2 (%)
results also stress this important concept. Nearly 36 per cent of
2/2 (100) the patients (19 of 53) had another urothelial tumor. In 42 per
4/5 (80) cent of these patients (8 of 19) an abnormality was present in
1/3 (33.3) the renal pelvis, such as carcinoma in situ (4 of 8). The remain-
1/3 (33.3)
0/4 (0) ing patients (11 of 19) had bladder tumors. Carcinoma in the
contralateral ureter occurred in 2 patients (3.7 per cent). This
incidence correlates well with the reported figure of 3 to 4 per
cent of the patients with upper tract tumors in whom malig-
nancy develops in the opposite kidney. 9
Another interesting finding was that only 2 of 19 patients
suffered additional new tumors after the ureteral lesion. Both
patients had a second urothelial carcinoma 2½ to 3 years after
the initial ureteral lesion was resected. The remaining 17 pa-
tients in group 2 either had a previous tumor or a simultaneous
lesion.
The over-all survival rate for patients at risk ~5 years is 50
per cent (23 of 46 cases). No difference in over-all outcome is
found according to group of patients. The 5-year rate for all
group l patients is 48.3 per cent (14 of 29) and for group 2 it is
47.7 per cent (8 of 17). Accordingly, depth of invasion is the
most important indicator of over-all results. The natural course
of the tumor is not affected by the finding of a second urothelial
carcinoma. In addition, the natural history of low stage ureteral
carcinomas is not influenced by the extent of surgical resection.
Regardless of the surgical procedure these patients have a good
prognosis. Hence, a more conservative surgical approach is
indicated for these patients with low stage tumors.
 REFERENC2S UnfottuT.. ately 1 it diffi_~ult Hie
L Richie, J. P .. m.c"'"IS"'""'" of ureteral turnors. In: invob18d renzJ u:::dt Releva:1t. r:nan2.gs1Yicut Ca.Inerot': :rns_de
Cancer. Edited G. Skinner and J. B. deKernion. the significant observatio:n. that a of the 60 vvitb ureteral
phia: W. B. ,,,,,m"P''' Co., chapt. 150, 1978. turnors haci maltiple ipsilateral turners the ureter o:r pelvis.;
}1_ direct rel2.t1c,m;l1Jp among tuffto-r grade) of invasion and s~1rvival
2. Batata, M. A., Whitmore, W. F., Jr., B. S., To:rita, N. and
Grabstald, H.: Primary carcinoma of the ureter: a prognostic has been :reported. (Also references 2 5 in article.)
study. Cancer, 35: 1626, 1975. It would seem that oniy if the authors a;:e able to assess r-eliably the
3. Hawtrey, C. E.: Fifty-two urete:ral carcinoma: a ipsilateral urothelium and determine that it, as well as the witnessed
clinical-pathologic 188, 1971. ureteral lesion, is low grade could the approach be individuai-
4. Beck, A. D., Heslin, J.E., W. A. and Garlick, W. B.: Primary ized. If not, the most conservative approach should err in the
tumors of the ureter: diagnosis and management. J-. Urn!., 102: direction of appropriate cancer
683, 1969. Kenneth
5. Bloom, l...J. A., \/idone, R. _A.. and Lytton, B.: Primary carcinoma of Department of
the ureter: a report of 102 new cases. J. Urol., H!3: 590, 1970. University School of lvf.edicine
6. McCarron, J. P. and Max-shall, V. F.: The survival of J1.1adison,
bladder tumors treated surgery: cornparative
and a recent series. J. 122: 322, 1979.
7. Twinem, F. P.: Primary tumors of the ureter. J.A.M.A., 163: 808,
1957. REPLY BY AUTHORS
8. Heney, N. M., Nocks, B. N., Daly, J. J., Blitzer, P.H. and Parkhurst,
E. C.: Prognostic factors in carcinoma of the ureter. J. Urol., 125: with the sugges-
632, 1981. tion that adjuvant stage ureteral
9. Cummings, K. B.: Nephroureterectomy: rationale in the manage- tumors may be beneficial.
ment of transitional cell carcinoma of the upper urinary tract. Recent upper tract mapping studies give support for less ag:,g:n\ss1ve
Urol. Clin. N. Amer., 7: 569, 1980. surgery in patients vvith low grade lesions.'·' In 30 specimens
good correlation with and In addition none of noninvasive
grnde I lesions or tumor in the remainder of the
EDITORIAL COMMENTS specimen. In contrast, all invasive
These authors further documentation of the importance of showed associated severe
e,o,cu,v,v"',~ stage the prognosis of patients with primary carcinoma II
the ureter. Additional impetus is given to a conservative associated with cytologic
approach i.n patients with low I would concur Accordingly,
use of conservative pr·oc:ectm·es with lovv grade (as
determined by brush "'"'"""" low stage tumors. This
excellent retrospective alsc •"Ar.tw,~c the need for more agvessive iesions. 1-Iowever, close follovvup
therapy in patients with stages C and D tumors. Radical nephroureter-
ectomy with regional lymphadenectomy should be used and adjuvant 1.
therapy with agents such as cis-platinurn should be considered.
Jerome P. Richie 2. H.M.,
tu:ffiors. U rol.j

3.

The authors have presented a large series of with ureteral

tumors and appropriately conclude that invasion is the most
important predictor of end results. n.,m,,u,m,u,_y, they appeal for a
conservative approach for patients with stage turr.1.ors.
The rationale departure from the classic radical nephrou:reter- 5. 1\JicCa.rron, J.P., EL and Gray,(}. F., Jr.: .'-',rd,ornqh
ectomy with bladder cuff is on the wuuw111~ considerations: of ne·Dllrc)rn,e1;erectom specimens uro-
l) necess:tty to preserve function and 2) cu,mµeu1u" evidence that - and cU.nical correlation. cT.
the re,co1~ntze,ct ureteral lesion is truly low stage.