International Urology and Nephrology 8 (2), pp. 99--106 (1976)

Malignant Tumours of the Renal Pelvis A. WOJEWSKI, T. ZAJACZKOWSKI Separment of Urology, Medical Academy, Szczecin, Poland (Received July 1, 1975)

A review of the clinical data of 22 patients with malignant tumours of the renal pelvis, accounting for 1 0 ~ of the total patient material of renal tumours, is presented. The earliest manifestation was prevalently haematnria, less often pain. In two thirds of the cases the diagnosis was established o n radiological evidence and in one third the process remained undetected until surgical exposure of the kidney. The diagnostic difficulties involved by tumours o f the renal pelvis and the value of the diagnostic methods are discussed. Nephroureterectomy with excision of a cuff of bladder was carried out in 15, nephrectomy in 5 cases. Three of the 22 patients have survived five years. Early diagnosis followed by total nephroureterectomy without delay may bring some improvement in the poor prognosis of these tumours. M a l i g n a n t t u m o u r s o f the renal pelvis differ in type f r o m those o r i g i n a t i n g in the renal p a r e n c h y m a . In the vast m a j o r i t y o f cases they are e p i t h e l i a l a n d arise f r o m the m u c o u s m e m b r a n e f o r m i n g t h e pelvocalyceal lining. A g r e a t deal of i n f o r m a t i o n on the behaviour, t h e r a p y a n d p r o g n o s i s o f these t u m o u r s has been p r o v i d e d d u r i n g the last two decades by studies o f Seth-Smith [6], T a y l o r [7], C u m m i n g s et al. [3] a n d others. The c o m p a r a t i v e l y low incidence o f this type o f turnout, on the one hand, a n d the diagnostic difficulties, on the other, seemed to justify a review o f o u r m a t e r i a l in the light o f o u r observations.

Material In the years 1 9 5 6 - 7 4 , 222 patients with renal n e o p l a s m s were treated, the turnouts having been o f pelveocalyceal origin in 22 cases. Twelve o f these patients were u n d e r t r e a t m e n t between 1956 a n d 1969, 12 patients in the last f o u r years, i.e. in 1 9 7 0 - 7 4 . A g e o f the patients r a n g e d between 25 a n d 70 years, the m a j o r i t y being between 50 a n d 70 years. T h e r e were 12 males a n d 10 females. The right k i d n e y was involved in 10, the left in 12 cases. The interval between the a p p e a r a n c e o f the first signs a n d a d m i s s i o n r a n g e d f r o m a few days to f o u r years. H a e m a t u r i a was present in 20, flank p a i n in 15 International Urology and Nephrology 8, 1976

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cases. The tumour was accessible to palpation in 9 cases. The sedimentation rate was increased in 11 cases. A considerable loss of body weight was reported by 6 patients. The excretory urogram revealed a non-excreting kidney in 13 cases, i.e. in the majority of the patients. In 6 cases the radiogram was indicative of a renal tumour (enlargement of the kidney, irregularity of its outlines, extensive deformity of calyces and pelvis), though not of its primary site. In 3 cases the urogram was suggestive of a pelvic origin (distended, poorly outlined renal pelvis, filling defect (Fig. 1). A coexistent intrapelvic calculus was found in 3 cases.

Fig. 1. Papillary carcinoma. Urography. Dilated, poorly outlined pelvis with filling defects

Retrograde pyelography was performed in 9 cases, urography showing a nonexcreting kidney in 6 and a poor and fragmentary filling of the renal pelvis in 3. The changes revealed by the retrograde pyelogram were typical of a pelvic tumour in 8 cases, of a renal tumour in one case. Renal angiography was made in four patients. In one case renal arteriography revealed a network of abnormal vessels in the upper and middle parts of the kidney. It showed minute reservoirs of contrast medium in the parenchymal phase, and deformity o f the renal outline as well as a loss of difference in opacity between the cortical and the medullary area in the nephrographic phase. In the second case the sharply delineated pathological vessels protruded from the projection of the International Urology and Nephrology

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Fig. 2. Papillary carcinoma. Selective angiography. In the projection of the pelvis there are pathological vessels. The model of the tumour is outlined by the course o f the segmental arteries

Fig. 3. The same case. Selective phlebography. Impaired patency of the lower segmental veins International Urology and Nephrology 8, 1976

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renal pelvis and the turnout was out/ined by the course o f the segmental arteries (Fig. 2). Selective renal phlebograplay (Fig. 3) showed obstruction of a segmental vein. Thrombosis, possibly of malignant nature, was suspected. On surgical exposure it became evident that the factor accounting for the obstruction of the vein was its compression by the tumour. In the third case with a non-functioning

Fig. 4. Papillary carcinoma. Selective arteriography, Renal arterial stenosis. In the projection of the dilated pelvis, conglomerate of pathological vessels. Dilatation of pelvic artery and capsular branches

kidney the arteriogram showed scanty ramifications of the intrarenal arteries, a poor opacification of the renal parenchyma and a loss of difference between the opacities o f the cortical and medullary areas. Hydronephrosis of obstructive origin was suspected. On exploration a papillary carcinoma o f pelvocalyceal origin was found. The following case deserves interest. F. S., a 61-year-old man, had been experiencing occasional painless haematuria for the last two years. Cystoscopy disclosed a sessile papilloma of the size of a large plum in the area of the left ureteral orifice. Urography showed a nonfunctioning left kidney. Surgery consisting in excision of the tumour with the left half of the bladder and reimplantation of the ureter was performed. Fifteen months later the patient presented with haematuria. Local recurrence of the tumour was ascertained on the evidence of cystoscopy and radiography. On the urogram the left kidney was not visualized. Selective renal arteriography (Fig. 4) revealed narrowing of the renal artery and its branches. In the projection o f the distended International Urology and Nephrology 8~ 1976

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pelvis a conglomerate of abnormal vessels was seen. The pelvis and capsular arterial branches were dilated. On renal phlebography (Fig. 5) filling was confined to the dorsal veins of arcuated shape, curving upward and tapering off toward the periphery. The far advanced stage of the tumour and the poor condition of the patient made surgery unwarrantable.

Fig. 5. T h e s a m e case. Selective p h l e b o g r a p h y . F i l l i n g c o n f i n e d to t h e d o r s a l v e i n

The results of radiography were confirmed by those of renography and renal scintigraphy performed in individual cases. Cytology of the urine revealed malignant cells in 2 out of 6 cases. In 4 cases the pelvic tumour was detected by chance, on surgery for other causes, i.e. for pelvic calculi in 2 cases, for hydronephrosis in one case. In the fourth case, six years after incision of a perirenal abscess, squamous cell carcinoma Table 1 M e t h o d of t r e a t m e n t a n d r e s u l t s

Surgical intervention

Nephroureterectomy Nephrectomy Pelvic wall r e s e c t i o n Conservative

Survival (years)

Histopathological diagnosis Papillary carcinoma

15 5 1 1

15 2 1

Squamous cell carcinoma

3

>5

>5

6 1

3

Died within 3 years

No information

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was detected in a fistula of poor healing tendency opening into the renal pelvis which had been the site of an impacted calculus for many years. Nephroureterectomy with excision of a cuff of bladder was carried out in 15 cases, nephrectomy and partial ureterectomy in 5 cases. Two patients of the last-named group had secondary ureterotomy with segmental resection of the bladder owing to local recurrences in the ureteral stump and the bladder. In tumours of a solitary kidney our surgical activities were confined to the excision of the tumour together with the pelvic wall. Microscopic study revealed papillary carcinoma in 18 and squamous cell carcinoma in 3 cases. Ten of the 22 patients are still alive, three having survived more than five years. Nine died within three years after operation and 2 are unaccounted for. In the group where the operation had been confined to nephrectomy none of the patients had survived the period of three years.

Discussion

In the material presented here malignant tumours of the renal pelvis account for 10 70 of those of the entire kidney. These tumours are prevalently papillary carcinomas, rarely squamous cell carcinomas. It is of interest that the incidence of tumours of the renal pelvis has been on the increase for the last years, as it has been confirmed by Wagenknecht and Wehling [8]. The earliest sign is generally haematuria. Pain is rarely an early sign, it occurs in a later stage. Other signs or symptoms, such as fever, increased sedimentation rate, elevation of blood pressure, weight loss and anaemia are less characteristic. Pelvic neoplasms may remain silent for a long time. Diagnosis of the tumour is difficult, particularly in the early stage. Even if the diagnosis of a renal tumour has been made before surgery, it is often the microscopic finding of the nephrectomy specimen which permits to identify its pelvic origin. Approximately 33 70 of these tumours owe their detection to surgery for other diseases. Coexisting calculosis may also cause a delay in diagnosis by sharing the presenting symptoms of the tumour, i.e. dull pain, renal colics and haematuria. The most reliable diagnostic indications are provided by X-ray studies, in the first place by excretion urography which may show filling defects, irregular outlines, displacement, compression of the renal pelvis and calyces. The most characteristic sign is, however, the intrapelvic filling defect confirmed by repeated urography. In case of large tumours compressing the renal parenchyma the affected kidney may fail to excrete the contrast or excretes it poorly in which case intrapelvic filling is indistinct or confined to irregular spots. This explains why excretion urography gave inconclusive results in nearly half of the cases. The finding of a non-excreting kidney calls for retrograde pyelography or for selective arteriography. Small pelvic tumours are, however, generally not visualized by arteriogInternational Urology and Nephrology 8, 1976

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raphy. Billewicz [1 ] calls attention to the dilatation o f the renal pelvic artery and its ramifications in case of pelvic tumours, an observation confirmed in the present cases too. Marczynska [5] advocates pneumopyelography if pyelography should prove inconclusive. Cancers of pelvic origin form no malignant thrombi in the large veins of the kidney. Some authors point to the applicability of pyeloendoscopy and pyeloradiography. Renography and renal scintigraphy supplement the radiological findings by providing information on the degree and extent of destruction of the renal parenchyma. Cytological study of the urine is often negative, and it is made often difficult by the massive amounts of red blood cells in the urine. It should be nonetheless performed in every case. Grace et al. [4] found malignant cells in 33 ~ of their cases with tumours of the renal pelvis. In neoplasms of the urinary bladder radiographic study of kidney and ureters is mandatory. Owing to neglect of this routine in one of our cases a turnout of the renal pelvis remained undetected until it was too late for nephrectomy. Today, the generally accepted treatment for tumours of the renal pelvis consists in nephroureterectomy with excision of a cuff of bladder. The operation is feasible with one or two incisions. In 2 out of 5 patients who had nephrectomy only, the operation was soon followed by a recurrence in the ureteral stump and bladder. In tumours of a solitary kidney surgical therapy has to be confined to the excision of the tumour. After the operation the patients should be checked cytoscopically at sixmonth intervals for possible recurrences in the bladder. Tumours of the renal pelvis generally carry a poor prognosis, depending on their histological structure and on the differentiation of their cells. Three of 21 patients have survived the operation for more than five years without any recurrence of the tumour. Taylor [7] reports survivals over five years in 8 out of 30 patients (23 ~ ) with papillary carcinoma of the renal pelvis. Squamous cell carcinomas are known for their extreme malignancy. In all three of the present cases the survivals were very short, the patients having died five to eighteen months after operation. There is only one five-year survival reported by Carlson [2]. Summing up, it is to be emphasized that suspicion of carcinoma of the renal pelvis calls for the concurrent use of every available diagnostic means including surgical exposure. The prognosis of these tumours has been hitherto very poor. Early diagnosis followed by nephroureterectomy offers the only chance for its improvement. References 1. Billewicz, O.: Varto~6 radiologicznego bandanie naczyniowege w guzach nerek. Gdansk 1967. 2. Carlson, H.: Squamous cell carcinoma of the renal pelvis, a five-year cure. J. Urol. 83, 812 (1960). International Urology and Nephrology 8, 1976

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3. Cummings, K., Correa, R., Gibbons, R., Stalt, H., Wheelis, R., Mason, J.: Renal pelvic tumours. J. Urol. 113, 158 (1975). 4. Grace, D., Taylor, W., Taylor, J., Winter, C.: Carcinoma of the renal pelvis: a 15-year review. J. Urol. 98, 566 (1968). 5. Marczynska, A.: Nowotwory brodawczakowate mied niczki nerkowej. UroL PoL 10, 81 (1956). 6. Seth-Smith, A.: Turnouts of the renal pelvis: Review of sixty-four cases. Brit. Y. Urol. 31, 265 (1959). 7. Taylor, W. :Tumours of the kidney pelvis. J. Urol. 8 , 452 (1959). 8. Wagenknecht, L., Wehling, Iff.: Wertigkeit diagnostischer Mal3nahmen bei NierenbeckenTumoren. Zschr. Urol. 67, 423 (1974).

International Urology and Nephrology 8, 1976

Malignant tumours of the renal pelvis.

International Urology and Nephrology 8 (2), pp. 99--106 (1976) Malignant Tumours of the Renal Pelvis A. WOJEWSKI, T. ZAJACZKOWSKI Separment of Urolog...
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