Pediatr Radiol (1992) 22:203-205
Pediatric Radiology 9 Springer-Verlag 1992
Renal cell carcinoma in childhood J. E. Kabala 1, J. Shield 2 and A. D u n c a n 1 1Department of Radiology and 2Department of Oncology, Bristol Hospital for Sick Children, St. Michael's Hill, Bristol, UK Received: 6 January 1992; accepted: 17 February 1992
Abstract. T h e imaging features of renal cell carcinoma in 4 y o u n g patients (age 7 to 14 years) are described. A high p r o p o r t i o n (75 % ) s h o w e d calcification on plain radiographs or c o m p u t e d t o m o g r a p h y (CT). B o t h patients w h o u n d e r w e n t C T s h o w e d well defined high density t u m o u r s which were also echogenic on ultrasound examination. These findings are significantly different to those m o s t c o m m o n l y seen on studies of the t u m o u r in adults.
Wilms t u m o u r is the c o m m o n e s t malignant a b d o m i n a l t u m o u r in childhood, usually presenting b e l o w the age of 5 years. R e n a l cell carcinoma, however, is occasionally enc o u n t e r e d but because of its rarity it m a y not be initially considered in the diagnosis. T h e imaging features of renal cell carcinoma in a d u l t h o o d are well d o c u m e n t e d but this is not the case in childhood; four cases are described below with particular emphasis on those findings that a p p e a r less c o m m o n l y in adulthood.
Case reports Case 1 7-year-old girl presenting with sudden onset of haematuria lasting four days. This had been preceeded by several weeks malaise associated with mild intermittent right loin and central abdominal pain. There was no other relevant medical or family history. On examination the patient was normotensive and the lower pole of the right kidney was thought to be palpable. An intravenous urogram (IVU) was essentially normal, showing only debatable increase in thickness of the cortex over the lower pole of the right kidney. Ultrasound examination revealed a well-defined 3 cm diameter echogenic lesion at the lower pole of the right kidney (Fig. 1). On CT the lesion was well-defined, homogenous and of higher density than the normal renal parenchyma. Following intravenous contrast medium the Fig.1. Casel; ultrasound of the right kidney showing a 3 cm diameter echogenic mass in the lower pole (cursors) Fig.2a,b. Case 1; CT scan through the right kidney showing a welldefined high density renal cell carcinoma (a) which enhances poorly with intravenous contrast (b)
204
Fig. 3 a,b. Case 2; IVU showing a large left lower pole mass, containing flecks of calcification on the plain film (a) and causing elevation of the lower pole calyces (b). Lateral deviation of the mid ureter is also seen on the contrast film, presumably related to para-aortic lymph node metastases Fig.4. Case 3; IVU showing large right upper pole mass with compression of the surrounding calyces Fig.5. Case3; Ultrasound of the right kidney showing a 4 c m diameter echogenic upper pole renal cell carcinoma l~/g.6 a,b. Case 3; CT scan through the kidneys showing a high density, partly calcified right renal mass (a) which enhances poorly with intravenous contrast medium (b)
lesion enhanced poorly and became of considerably lower density than the normal kidney substance (Fig.2a, b). A radical nephrectomy was performed, histological examination revealing a renal adenocarcinoma; a single hilar lymph node was found to be involved with metastatic tumour making the carcinoma overall stage III b on Robson's classification [1]. At three months follow-up the patient remains well and free of tumour.
Case 2 14-year-old girl presenting with a urinary tract infection. There was no previous family or medical history except urinary tract infections 10 years beforehand, investigated with an IVU and micturating cystourethrogram which revealed left vesico-ureteric reflux to the left pelvic brim. On examination a large, left upper quadrant mass was discovered. IVU (Fig. 3 a, b) revealed a large mass at the lower pole of the left kidney, elevating the pelvicalyceal system and seen to contain flecks of calcification on the preliminary film. The mid-ureter on the left was also seen to be markedly angled laterally. CF facilities were unavailable at that time. At laparotomy a large (15 cm diameter), lower pole renal tumour was encountered which later was shown histologically to be a renal adenocarcinoma. A second 10 cm diameter mass was found inferior to the kidney and shown to be a group of para-aortic lymph nodes involved with tumour, making the carcinoma overall stage IIIb. The patient underwent a left radical nephrectomy and lymphadenectomy followed by local radiotherapy and chemotherapy. Unfortunately 12 months later the patient developed spinal metastases and locally recurrent tumour. Further chemotherapy produced a remarkably prolonged remission but with ultimate death 38 months from the time of the original diagnosis.
205 Table 1. Clinical features Case
Age
Presenting features
Pathological stage
Survival
1
7
Haematuria
III b
No recurrence at 12 months
2
14
Mass
III b
38 months
3
13
Haematuria
II
No recurrence at 54 months
4
10
Haematuria
II
Unknown
Table 2. Radiological features Case
Calcification
Ultrasound
CT
1
None demonstrated
3 cm echogenic lesion
Homogenous high density; poorly enhancing
2
Plain film
-
3
CT
4 cm echogenic lesion
High density; poorly enhancing
4
Plain film
-
-
Case 3 13-year-old male presenting with 2 episodes of haematuria over a period of one week, each associated with loin pain. No other relevant family or medical history. On examination the patient was normotensive and no masses were palpable in the abdomen. IVU revealed a large mass in the upper pole of the right kidney with the upper pole calyces distorted around it (Fig. 4). Ultrasound showed a well defined 4 cm diameter echogenic mass in the upper pole of the right kidney (Fig. 5). CT showed the mass was of slightly higher density than normal renal parenchyma and contained at least one area of cmwilinear calcification. A thin low density rim was discernible, no doubt due to the surrounding compressed calyces. The lesion enhanced poorly with intravenous contrast medium, becoming less dense than normal renal parenchyma (Fig. 6 a,b). There was no evidence of vascular involvement. A radical nephrectomy was performed and a stage II adenocarcinoma of the kidney was removed i.e. tumour breaching the capsule and involving the perirenal fat but without evidence of any further spread (vascular, lymphatic or metastatic). At 54 months the patient is alive and well without evidence of recurrent tumour.
Case 4 lO-year-old boy presenting with haematuria and right loin and right iliac fossa pain. No previous medical or family history. A right loin mass was palpable. IVU showed a large middle and lower pole mass which was seen on the preliminary film to contain calcification. CT was unavailable at that time. A large stage II adenocarcinoma of the kidney was found at operation at which time a radical nephrectomy was performed. Unfortunately the patient was lost to follow-up and outcome is therefore unknown. The major clinical and imaging points are summarised in tables 1 and 2. Discussion A l t h o u g h well r e c o g n i s e d r e n a l cell c a r c i n o m a is an unc o m m o n t u m o u r in c h i l d h o o d . Less t h a n 0.5 % a r e s e e n in p a t i e n t s b e l o w t h e age o f 20 y e a r s a n d the f r e q u e n c y of r e n a l cell c a r c i n o m a c o m p a r e d with W i l m s t u m o u r in c h i l d h o o d is a r o u n d 2-6.6 % [2M]. M e a n a g e of p r e s e n t a tion is 11-12 years, m o s t c o m m o n l y w i t h h a e m a t u r i a a n d l o i n / a b d o m i n a l p a i n [5]. A s with the t u m o u r in a d u l t h o o d the o n l y effective t r e a t m e n t is r a d i c a l n e p h r e c t o m y with regional adenectomy; neither chemotherapy or radiother-
apy produce a sustained response. Consequently the prognosis is similar to t h a t in a d u l t s a n d p o o r c o m p a r e d with W i l m s t u m o u r , b e i n g a r o u n d 5 2 - 5 6 % at 5 y e a r s [4-6]. C o n s i d e r i n g o n l y locally c o n f i n e d t u m o u r s , h o w e v e r , t h e p r o g n o s i s is m u c h better, a r o u n d 80 % at 10 y e a r s [2]. A l t h o u g h its b i o l o g i c a l b e h a v i o u r is s i m i l a r to t h e a d u l t t u m o u r , the r a d i o l o g i c a l a p p e a r a n c e s in the cases des c r i b e d h e r e s h o w s o m e significant differences. Classically t h e a d u l t r e n a l cell c a r c i n o m a o n C T is i l l - d e f i n e d a n d low density; i s o d e n s e o r h y p o d e n s e c o m p a r e d to n o r m a l r e n a l p a r e n c h y m a [7-9[. I n b o t h o f the p a t i e n t s w h o u n d e r w e n t C T s c a n n i n g in this c u r r e n t series t h e c a r c i n o m a a p p e a r e d i l l - d e f i n e d a n d high density. C a l c i f i c a t i o n can b e s e e n in 5 - 1 0 % of a d u l t r e n a l cell c a r c i n o m a s [10] b u t was d e m o n s t r a t e d in two of t h e cases d e s c r i b e d a b o v e on p l a i n film a n d in a t h i r d on CT, a h i g h e r t h a n e x p e c t e d p r o p o r t i o n (75 % ). T h e u l t r a s o u n d a p p e a r a n c e s of a d u l t r e n a l cell carcinom a a r e v a r i a b l e . 44 % a r e of similar e c h o g e n i c i t y to n o r m a l r e n a l p a r e n c h y m a , 35 % a r e h y p o - e c h o i c a n d o n l y 2 1 % h y p e r - e c h o i c [11]. S o m e w o r k e r s h a v e s u g g e s t e d t h a t h y p e r e c h o g e n i c i t y m a y b e a s s o c i a t e d with h y p e r v a s c u l a r t u m o u r s [12]. B o t h t h e p a t i e n t s w h o u n d e r w e n t u l t r a s o u n d in this series h a d well d e f i n e d h y p e r - e c h o i c tum o u r s a n d it is i n t e r e s t i n g to n o t e t h a t the s a m e t u m o u r s o n C T s h o w e d p o o r e n h a n c e m e n t suggesting t h e y w e r e n o t h y p e r v a s c u l a r lesions. References I. Robson JR, Churchill BM, Anderson W (1969) The results of radical nephrectomy for renal cell carcinoma. J Uro1101: 297-301 2. Castellanos RD, Aron BS, Evans AT (1974) Renal adenocarcinoma in children: incidence, therapy and prognosis. J Urol 111: 534-537 3. Cassady JR, Filler R, Jaffe N, Vawter G (1974) Carcinoma of the kidney in children. Radiology 112:691-693 4. Booth CM (1986) Renal parenchymal carcinoma in children. Br J Surg 73:313-317 5. Raney RB, Palmer N, Sutow WW, Baum E, Ayala A (1983) Renal cell carcinoma in children. Med Paediatr Onco111: 91-98 6. Lack EE, Cassady JR, Sallan SE (1985) Renal cell carcinoma in childhood and adolescence: clinical and pathological study of 17 cases. J Uro1133: 822-828 7. Hattery RH, Williamson B, Stephens DH, Sheedy PF, Hartman GW (1977) Computed tomography of renal abnormalities. Radiol Clin North Am 15:401418 8. Coleman BG, Arger PH, Mintz MC, Pollack HM, Banner MP (1984) Hyperdense renal masses: A computed tomographic dilemma. AJR 143:291-294 9. Levine E, Huntrakoon M, Wetzel LH (1989) Small renal neoplasms:clinical,pathologicalandimagingfeatures.AJR153:69-73 10. Abrams H J, Buchbinder MI, Sutton AP (1979) Renal cell carcinoma in adolescents. J Uro1121:92-94 11. Coleman BG, Arger PH, Mulhern CT, Pollack HM, Banner MR Arensen RL (1980) Gray-scale sonographic spectrum of hypernephromas. Radiology 137:757-765 12. Ladwig SH, Jackson D, Older RA, Morgan CL (1981) Ultrasonic, angiographic and pathologic correlation of non-cystic appearing renal masses. Urology 17:204-209 Dr. J. E. Kabala Department of Clinical Radiology Bristol Royal Infirmary Marlborough Street Bristol BS1 3NU, UK
Pediatric Radiology 9 Springer-Verlag 1992
Renal cell carcinoma in childhood J. E. Kabala 1, J. Shield 2 and A. D u n c a n 1 1Department of Radiology and 2Department of Oncology, Bristol Hospital for Sick Children, St. Michael's Hill, Bristol, UK Received: 6 January 1992; accepted: 17 February 1992
Abstract. T h e imaging features of renal cell carcinoma in 4 y o u n g patients (age 7 to 14 years) are described. A high p r o p o r t i o n (75 % ) s h o w e d calcification on plain radiographs or c o m p u t e d t o m o g r a p h y (CT). B o t h patients w h o u n d e r w e n t C T s h o w e d well defined high density t u m o u r s which were also echogenic on ultrasound examination. These findings are significantly different to those m o s t c o m m o n l y seen on studies of the t u m o u r in adults.
Wilms t u m o u r is the c o m m o n e s t malignant a b d o m i n a l t u m o u r in childhood, usually presenting b e l o w the age of 5 years. R e n a l cell carcinoma, however, is occasionally enc o u n t e r e d but because of its rarity it m a y not be initially considered in the diagnosis. T h e imaging features of renal cell carcinoma in a d u l t h o o d are well d o c u m e n t e d but this is not the case in childhood; four cases are described below with particular emphasis on those findings that a p p e a r less c o m m o n l y in adulthood.
Case reports Case 1 7-year-old girl presenting with sudden onset of haematuria lasting four days. This had been preceeded by several weeks malaise associated with mild intermittent right loin and central abdominal pain. There was no other relevant medical or family history. On examination the patient was normotensive and the lower pole of the right kidney was thought to be palpable. An intravenous urogram (IVU) was essentially normal, showing only debatable increase in thickness of the cortex over the lower pole of the right kidney. Ultrasound examination revealed a well-defined 3 cm diameter echogenic lesion at the lower pole of the right kidney (Fig. 1). On CT the lesion was well-defined, homogenous and of higher density than the normal renal parenchyma. Following intravenous contrast medium the Fig.1. Casel; ultrasound of the right kidney showing a 3 cm diameter echogenic mass in the lower pole (cursors) Fig.2a,b. Case 1; CT scan through the right kidney showing a welldefined high density renal cell carcinoma (a) which enhances poorly with intravenous contrast (b)
204
Fig. 3 a,b. Case 2; IVU showing a large left lower pole mass, containing flecks of calcification on the plain film (a) and causing elevation of the lower pole calyces (b). Lateral deviation of the mid ureter is also seen on the contrast film, presumably related to para-aortic lymph node metastases Fig.4. Case 3; IVU showing large right upper pole mass with compression of the surrounding calyces Fig.5. Case3; Ultrasound of the right kidney showing a 4 c m diameter echogenic upper pole renal cell carcinoma l~/g.6 a,b. Case 3; CT scan through the kidneys showing a high density, partly calcified right renal mass (a) which enhances poorly with intravenous contrast medium (b)
lesion enhanced poorly and became of considerably lower density than the normal kidney substance (Fig.2a, b). A radical nephrectomy was performed, histological examination revealing a renal adenocarcinoma; a single hilar lymph node was found to be involved with metastatic tumour making the carcinoma overall stage III b on Robson's classification [1]. At three months follow-up the patient remains well and free of tumour.
Case 2 14-year-old girl presenting with a urinary tract infection. There was no previous family or medical history except urinary tract infections 10 years beforehand, investigated with an IVU and micturating cystourethrogram which revealed left vesico-ureteric reflux to the left pelvic brim. On examination a large, left upper quadrant mass was discovered. IVU (Fig. 3 a, b) revealed a large mass at the lower pole of the left kidney, elevating the pelvicalyceal system and seen to contain flecks of calcification on the preliminary film. The mid-ureter on the left was also seen to be markedly angled laterally. CF facilities were unavailable at that time. At laparotomy a large (15 cm diameter), lower pole renal tumour was encountered which later was shown histologically to be a renal adenocarcinoma. A second 10 cm diameter mass was found inferior to the kidney and shown to be a group of para-aortic lymph nodes involved with tumour, making the carcinoma overall stage IIIb. The patient underwent a left radical nephrectomy and lymphadenectomy followed by local radiotherapy and chemotherapy. Unfortunately 12 months later the patient developed spinal metastases and locally recurrent tumour. Further chemotherapy produced a remarkably prolonged remission but with ultimate death 38 months from the time of the original diagnosis.
205 Table 1. Clinical features Case
Age
Presenting features
Pathological stage
Survival
1
7
Haematuria
III b
No recurrence at 12 months
2
14
Mass
III b
38 months
3
13
Haematuria
II
No recurrence at 54 months
4
10
Haematuria
II
Unknown
Table 2. Radiological features Case
Calcification
Ultrasound
CT
1
None demonstrated
3 cm echogenic lesion
Homogenous high density; poorly enhancing
2
Plain film
-
3
CT
4 cm echogenic lesion
High density; poorly enhancing
4
Plain film
-
-
Case 3 13-year-old male presenting with 2 episodes of haematuria over a period of one week, each associated with loin pain. No other relevant family or medical history. On examination the patient was normotensive and no masses were palpable in the abdomen. IVU revealed a large mass in the upper pole of the right kidney with the upper pole calyces distorted around it (Fig. 4). Ultrasound showed a well defined 4 cm diameter echogenic mass in the upper pole of the right kidney (Fig. 5). CT showed the mass was of slightly higher density than normal renal parenchyma and contained at least one area of cmwilinear calcification. A thin low density rim was discernible, no doubt due to the surrounding compressed calyces. The lesion enhanced poorly with intravenous contrast medium, becoming less dense than normal renal parenchyma (Fig. 6 a,b). There was no evidence of vascular involvement. A radical nephrectomy was performed and a stage II adenocarcinoma of the kidney was removed i.e. tumour breaching the capsule and involving the perirenal fat but without evidence of any further spread (vascular, lymphatic or metastatic). At 54 months the patient is alive and well without evidence of recurrent tumour.
Case 4 lO-year-old boy presenting with haematuria and right loin and right iliac fossa pain. No previous medical or family history. A right loin mass was palpable. IVU showed a large middle and lower pole mass which was seen on the preliminary film to contain calcification. CT was unavailable at that time. A large stage II adenocarcinoma of the kidney was found at operation at which time a radical nephrectomy was performed. Unfortunately the patient was lost to follow-up and outcome is therefore unknown. The major clinical and imaging points are summarised in tables 1 and 2. Discussion A l t h o u g h well r e c o g n i s e d r e n a l cell c a r c i n o m a is an unc o m m o n t u m o u r in c h i l d h o o d . Less t h a n 0.5 % a r e s e e n in p a t i e n t s b e l o w t h e age o f 20 y e a r s a n d the f r e q u e n c y of r e n a l cell c a r c i n o m a c o m p a r e d with W i l m s t u m o u r in c h i l d h o o d is a r o u n d 2-6.6 % [2M]. M e a n a g e of p r e s e n t a tion is 11-12 years, m o s t c o m m o n l y w i t h h a e m a t u r i a a n d l o i n / a b d o m i n a l p a i n [5]. A s with the t u m o u r in a d u l t h o o d the o n l y effective t r e a t m e n t is r a d i c a l n e p h r e c t o m y with regional adenectomy; neither chemotherapy or radiother-
apy produce a sustained response. Consequently the prognosis is similar to t h a t in a d u l t s a n d p o o r c o m p a r e d with W i l m s t u m o u r , b e i n g a r o u n d 5 2 - 5 6 % at 5 y e a r s [4-6]. C o n s i d e r i n g o n l y locally c o n f i n e d t u m o u r s , h o w e v e r , t h e p r o g n o s i s is m u c h better, a r o u n d 80 % at 10 y e a r s [2]. A l t h o u g h its b i o l o g i c a l b e h a v i o u r is s i m i l a r to t h e a d u l t t u m o u r , the r a d i o l o g i c a l a p p e a r a n c e s in the cases des c r i b e d h e r e s h o w s o m e significant differences. Classically t h e a d u l t r e n a l cell c a r c i n o m a o n C T is i l l - d e f i n e d a n d low density; i s o d e n s e o r h y p o d e n s e c o m p a r e d to n o r m a l r e n a l p a r e n c h y m a [7-9[. I n b o t h o f the p a t i e n t s w h o u n d e r w e n t C T s c a n n i n g in this c u r r e n t series t h e c a r c i n o m a a p p e a r e d i l l - d e f i n e d a n d high density. C a l c i f i c a t i o n can b e s e e n in 5 - 1 0 % of a d u l t r e n a l cell c a r c i n o m a s [10] b u t was d e m o n s t r a t e d in two of t h e cases d e s c r i b e d a b o v e on p l a i n film a n d in a t h i r d on CT, a h i g h e r t h a n e x p e c t e d p r o p o r t i o n (75 % ). T h e u l t r a s o u n d a p p e a r a n c e s of a d u l t r e n a l cell carcinom a a r e v a r i a b l e . 44 % a r e of similar e c h o g e n i c i t y to n o r m a l r e n a l p a r e n c h y m a , 35 % a r e h y p o - e c h o i c a n d o n l y 2 1 % h y p e r - e c h o i c [11]. S o m e w o r k e r s h a v e s u g g e s t e d t h a t h y p e r e c h o g e n i c i t y m a y b e a s s o c i a t e d with h y p e r v a s c u l a r t u m o u r s [12]. B o t h t h e p a t i e n t s w h o u n d e r w e n t u l t r a s o u n d in this series h a d well d e f i n e d h y p e r - e c h o i c tum o u r s a n d it is i n t e r e s t i n g to n o t e t h a t the s a m e t u m o u r s o n C T s h o w e d p o o r e n h a n c e m e n t suggesting t h e y w e r e n o t h y p e r v a s c u l a r lesions. References I. Robson JR, Churchill BM, Anderson W (1969) The results of radical nephrectomy for renal cell carcinoma. J Uro1101: 297-301 2. Castellanos RD, Aron BS, Evans AT (1974) Renal adenocarcinoma in children: incidence, therapy and prognosis. J Urol 111: 534-537 3. Cassady JR, Filler R, Jaffe N, Vawter G (1974) Carcinoma of the kidney in children. Radiology 112:691-693 4. Booth CM (1986) Renal parenchymal carcinoma in children. Br J Surg 73:313-317 5. Raney RB, Palmer N, Sutow WW, Baum E, Ayala A (1983) Renal cell carcinoma in children. Med Paediatr Onco111: 91-98 6. Lack EE, Cassady JR, Sallan SE (1985) Renal cell carcinoma in childhood and adolescence: clinical and pathological study of 17 cases. J Uro1133: 822-828 7. Hattery RH, Williamson B, Stephens DH, Sheedy PF, Hartman GW (1977) Computed tomography of renal abnormalities. Radiol Clin North Am 15:401418 8. Coleman BG, Arger PH, Mintz MC, Pollack HM, Banner MP (1984) Hyperdense renal masses: A computed tomographic dilemma. AJR 143:291-294 9. Levine E, Huntrakoon M, Wetzel LH (1989) Small renal neoplasms:clinical,pathologicalandimagingfeatures.AJR153:69-73 10. Abrams H J, Buchbinder MI, Sutton AP (1979) Renal cell carcinoma in adolescents. J Uro1121:92-94 11. Coleman BG, Arger PH, Mulhern CT, Pollack HM, Banner MR Arensen RL (1980) Gray-scale sonographic spectrum of hypernephromas. Radiology 137:757-765 12. Ladwig SH, Jackson D, Older RA, Morgan CL (1981) Ultrasonic, angiographic and pathologic correlation of non-cystic appearing renal masses. Urology 17:204-209 Dr. J. E. Kabala Department of Clinical Radiology Bristol Royal Infirmary Marlborough Street Bristol BS1 3NU, UK
