flin. Radiol. (1979) 30, 197-201

Non-Function in Nephroblastoma (Wilms's Tumour): A Report on the Excretory Urography of Nine Cases B. J- CREMIN

6roote Schuur and R e d Cross ChiMren 's Hospitals, University o f Cape Town, South Africa Non,function o f a kidney invaded b y nephroblastoma has n o t been extensively documented and may be featured only when a relatively large series is reviewed. It occurred in nine (15.5%) o f 58 cases seen over an eight year period. Two o f these nine cases had bilateral involvement and one showed curvilinear calcification. The mechanism of non-function appears to be due to replacement of most o f the kidney substance b y tumour, and this may be augmented b y invasion o f the renal pelvis causing malignant hydronephrosis. Ultrasonography has become increasingly utilised in confirming the diagnosis o f renal tumours, but arteriography may still be the critical examination in the 'unusual' case. Nephroblastoma is a c o m m o n neoplasm o f early childhood but when all age groups are taken into account it is also the second most c o m m o n form o f malignant disease o f the kidneys (Davidson, 1977). Its main urographic features are an intrarenal mass and these were reviewed in Clinical Radiology by Cope et al. (1972). These authors noted that though ~npaired renal function occurred in 22% (7 out of 32 cases), non-function occurred only once in their series, and they considered this was a rare phenomenon. Our experience with nephroblastoma is that failure for visualisation even with high dose urography techniques m a y n o t be unusual. An account

of these cases and described.

the

probable mechanism

is

MATERIAL AND METHODS

During the eight-year period 1969 76, 58 cases of nephroblastoma were seen at the Red Cross Children's Hospital, Cape Town. The excretory urogram failed to visualise the affected kidney in nine cases (15.5%). The pertinent features o f these cases are given in Table 1 and the patients (Cases 2 and 9) who had bilateral involvement are described in fuller detail later. Five of the children are at present alive and

Table 1

Caseno.

Age at presentation

Date of admission and survival

Side involved

Pathology

1. M.W.

8 years

May 1970, a/w

Left

2. M.D.J.

2 years

3. M.B.

2 years

Bilateral nonfunction on left Right

4, N.Z. L L.I~I. 6. G.S.

7 years 1 year 8 months 2 years

January 1971, died 10 days after admission January 1971, died March 1971 January 1973, a/w September 1973, a/w March 1974, a/w

Right

7. S.I.

4 years

Right

8. ~I.K.

1 year

October 1974, died March 1976 October 1976, a/w

Right

9. L.B.

2 years

November 1976, died four days after admission

Bilateral nonfunction both sides

Tumom replacing most of kidney except for small amount at lower pole. Renal pelvis involved, renal vein and inferior vena cava infiltrated Left kidney replaced by tumour mass. Right kidney well defined large turnout Turnout replacing kidney_ Inferior vena cava invaded Total replacement of kidney by tumour Large tumour involving majority of kidney and pelvis Large tumour with haemorrhagic and necrotic markings that infiltrated through pseudocapsule win little remaining renal tissue Total kidney replacement and encroachment on pelvis by turnout Kidney largely replaced by tumour which encroached on pelvis Total replacement of left kidney by tumour. Extensive involvement of right kidney and pelvis with blockage of ureter and malignant hydronephrosis

Right Left

198

CLINICAL RADIOLOGY

well. N o n e presented with radiological evidence o f metastases, b u t in two, extensive lung secondaries were present at autopsy. Macroscopic h a e m a t u r i a was n o t e d only twice. All unilateral cases had n o r m a l sized contralateral kidneys, w i t h the exception o f Case 1 in which the u n a f f e c t e d k i d n e y was enlarged by 2.5 s.d. ( m e t h o d o f E k l 6 f and Ringertz, 1976). Calcification was present in the n o n - f u n c t i o n i n g k i d n e y in Case 9, and this was t h e o n l y t i m e it was n o t e d in the series. A r t e r i o g r a p h y was p e r f o r m e d in seven of the cases and showed thin, angular neovascularity w i t h large areas of avascularity typical o f n e p h r o b l a s t o m a in this age group. A notable feature was l a c k o f opacification of the renal c o r t e x during perfusion and n e p h r o g r a m phases. Inferior v e n a c a v o g r a p h y b y catheterisation o f t h e femoral vein was p e r f o r m e d in three cases (3, 7 and 8). The results were equivocal and in only one (Case 3) was o b s t r u c t i o n f o u n d at surgery. Ultrasound was available for the last two cases only and showed enlarged kidneys w i t h c o m b i n e d solid and cystic patterns. T h e features o f the t w o cases w i t h bilateral involvement are presented below. M.D.J. A black female of two years and three months was referred to the hospital with a history of lassitude and failure to grow. The mother had felt a left abdominal mass two months prior to admission. The child was marasmic, weighed only 12kg and had bilateral palpable loin masses, larger on the left. The excretory urogram (Fig. 1) showed non-function

Fig. 1 - Bilateral nephroblastoma. Excretory urogram showing typical deformity of the pelvi-calyceal system on the right with no visualisation of the system on the left.

Fig. 2 - Control film showing bilateral renal masses with calcification on the right (arrows). of the left kidney and a large intrarenal lesion on the right. The child was considered too sick for radical surgery or radiotherapy, and although an initial course of vincristine was commenced the child died within a week. Autopsy showed that both kidneys were extensively involved by haemorr-

Fig. 3 - Fifteen minute tomogram of excretory urograrn. No function on the left and faint renal function with.,lucent areas on the right.

NON-FUNCTION

IN N E P H R O B L A S T O M A

Fig, 4 - Sagittal u l t r a s o u n d of right kidney showing r o u n d ~e~hoic areas confirming t h e hydronephrosis.

hagic, necrotic nephroblastoma. On t h e left side no recognisable kidney tissue remained whilst o n the right t h e central portion of t h e kidney was occupied by t u m o u r . A small amount of blood-stained ascites was present and t h e descendhag colon and pancreas were adherent to t h e left kidney. There was no glandular enlargement or evidence o f venous thrombosis.

Pig, 5 - Film taken in the late arterial phase o f a left selective arteriogram. No renal cortical p a r e n c h y m a is seen, the vessels are stretched and there is some neoplastic circulation (arrows).

199

L B . A black male aged two years presented with a history o f total anuria for 12 days. Prior to transfer-to our hospital h e had been maintained on peritoneal dialysis. On examination the child had generahsed o e d e m a and there were hard masses palpable in each flank. A small a m o u n t of bloodstained urine was obtained on catheterisation. The plain radiograph showed bilateral renal masses with some faint curvilinear calcification in the right renal area (Fig. 2). After intravenous injection o f 3 0 m l Conray 325 (sodium iothalamate 54% w]v), t h e 5 m i n radiograph showed n o visualisation of t h e collecting systems. At 15 m i n t o m o g r a p h y was performed. O n the left, no f u n c t i o n was seen; o n the right, there was a very faint rim o f p a r e n c h y m a surrounding lacent areas, suggesting the appearance o f hydronephrosis or cysts (Fig. 3). A voiding cysto-urethrogram was normal. Ultrasonography showed an enlarged left kidney with echoes f r o m solid interfaces; the right side had a similar appearance but greater dilatation o f the collecting system with a pattern that suggested t u m o u r or debris (Fig. 4). At this stage a diagnosis o f obstructed kidneys from either inflammatory lesions such as tuberculosis or x a n t h o g r a n u l o m a t o u s pyelonephritis, or neoplastic disease, was made. Bilateral selective renal arteriography was p e r f o r m e d and o n the left side it showed stretched renal vessels surrounding an essentially avascular kidney. No opacified renal cortex was n o t e d b u t neoplastic vessels were faintly visualised in the mid renal area (Fig. 5). On t h e right side neoplastic circulation was evident (Fig. 6) and some remnants of cortical tissue were seen.

Fig. 6 - Right selective arteriogram showing t h e presence of renal cortex and neoplastic circulation.

200

CLINICAL RADIOLOGY

Fig. 8 - Specimen of right kidney showing large tum0ur blocking renal pelvis.

DISCUSSION

Fig. 7 - Antegrade pyelogram showing hydronephrosis and hydroureter from tumour invasion of renal pelvis and obstruction of lower ureter. A diagnosis of extensive malignancy replacing and obstructing both kidneys was considered likely. In view of the serious condition of the child and the necessity for an exact diagnosis, a percutaneous antegrade pyelogram was performed on the right side. This demonstrated a hydronephrosis and hydroureter and showed obstruction in the collecting system (Fig. 7). Biopsy at a formal nephrostomy confirmed a nephroblastoma. All treatment was stopped and the patient died within 24h. Post-mortem examination revealed an almost total replacement of the left kidney by tumour mass and extensive involvement of the renal pelvis by turnout on the right side (Fig. 8).

The only other series which have noted non, function in nephroblastomas have been by Lalli et al. (1966) and Westra et al. (1967). Our cases were examined using relatively large doses o f contrast media ( 2 - 3 ml/kg) and the results are comparable with the 9.7% (3 out of 31 cases) seen at the Karolinska Sjukhuset during 1 9 5 2 - 6 6 by Lalli et al. Comparison with the high figure o f 31% (9 out of 29 cases) published by Westra et al. may not be valid as only 29 of their 46 cases collected from 1936-60 had intravenous urography and half the cases were diagnosed by retrograde pyelography. Their artide was mainly concerned with treatment and prognosis and they found a poor prognosis in non-functioning cases, which has not been borne out by our cases (vide infra). The mechanism o f non-function of the kidney on excretory urography may be one of the following: (a) complete tissue replacement by the tumour. (b) pelvi-ureteric invasion from within the pelvis causing a malignant hydronephrosis. (c) renal vein occlusion. In our cases the pathological reports indicated that the first mechanism was the major causative factor and this would account for the lack of renal cortical opacification seen during arteriography. The second mechanism involving pelvic obstruction was also seen and previous reports (Engel, 1976; Poole and Viamonte, 1970) have indicated that large turnouts may either completely replace renal tissue, or break

NON-FUNCTION IN NEPHROBLASTOMA

20]

inwards to fill and obstruct the entire collecting Acknowledgement. We thank Dr R. L. Golding for systems. Evidence o f venous obstruction was found in compiling the data for Table 1 and his assistance in per0nly two cases (Cases 1 and 3) but in these cases forming the arteriography in the last two cases. extensive tissue destruction by t u m o u r was also present- The concept that non-function is mainly due REFERENCES to parenchymal replacement and destruction differs Berdon, W. E., Baker, D. H. & Santulli, T. V. (1967). Factors from the commonly held view that it is due to renal producing spurious obstruction of the inferior vena cava vein occlusion (Gordon and Ross, 1977). in infants and children with abdominal turnouts. Radiology, 88, 111-116. The value of inferior venacavography m a y be doubtful in these cases; correct interpretation is Cremin, B. J. & Kaschula, R. O. C. (1972). Arteriography of Wilms's turnout - the results of 13 cases and comparison difficult (Berdon et al.. 1967) and our experience has to renal dysplasia. British Journal of Radiology, 45, 415supported this even when direct femoral catheteri422. sation was used. Cope, J. R., Roylance, J. & Gordon, I. R. S. (1972). The radiologic features of Wilms's tumour. Clinical Radiology, The use o f arteriography m a y also be queried, but 23,331-339. during a trial period in 1 9 7 0 - 7 4 pre-operative radioDavidson, A. J. (1977). Radiologic Diagnosis of Renal therapy was part o f our Wilms's tumour protocol, so Parenchymal Disease, p. 215. W. B. Saunders Co., that arteriography (Cremin and Kaschula, 1972) was Philadelphia. performed in all cases with uncommon features. We Ekl6f, O. & Ringertz, H. (1976). Kidney size in children: a method of assessment_ Acta Radiologica, 17, 617-625. do not now consider non-visualisation to be an Engel, R. M. E. (1976). Unusual presentation of Wilms's unusual feature and if required, ultrasound would be tumour. Urology, VIII, 288-289. the preferred subsequent examination. Gordon, I. R. S. & Ross, F. G. M. (1977). Diagnostic RadioArteriography still has a place in the difficult logy in Paediatrics, p. 269, Butterworths, London. Karabus, C. (1978). Personal communication. diagnostic problem and in Case 9 it was the first Lalli, A.F., Ahstr6m, L., Ericcson, N. O. & Rudhe, U. (1966). investigation to indicate malignancy. Nephroblastoma (Wilms's tumour): urographic diagnosis 'Non-function' should be regarded as a late compliand prognosis. Radiology, 87, 495-500. cation o f nephroblastoma. It does not necessarily Poole, C. A. & Viamonte, M. (1970). Unusual renal masses in indicate a bad prognosis as although the figures are the pediatric age group. American Journal of Roentgenology, 109,368-379. small for any valid conclusion the three year survival Westra, P., Kieffer, S. A. & Mosser, D. G. (1967). Wilms's rate of 57% (four out of seven cases) is comparable to turnout. A summary of 25 years of experience before that of the total series during the relevant period acinomycin-D. American Journal of Roentgenology, 100, 1969-74 (Karabus, 1978). 214-221.

Non-function in nephroblastoma (Wilms's tumour): a report on the excretory urography of nine cases.

flin. Radiol. (1979) 30, 197-201 Non-Function in Nephroblastoma (Wilms's Tumour): A Report on the Excretory Urography of Nine Cases B. J- CREMIN 6ro...
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