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WILMS TUMOR IN A PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY
Vol. 158, 1926-1927,November 1997 Printed in U.S.A.
WILMS TUMOR IN A PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY ANTONIO GONCALVES DE OLIVEIRA-FILHO, MARfLIA HOJAIJ CAFWALHO, LOURENCO SBRAGIA-NETO, M h C I O LOPES MIRANDA, JOAQUIM MURRAY BUSTORFF-SILVA AND EDISON RISSATO DE OLIVEIRA From the Departments of Surgery and Anesthesiology, Boldrini’s Children Cancer Center and Department of Pediatric Surgery, University of Campinas, Campinas, Brazil
KEYWORDS:kidney, polycystie;Wilms’ tumor; prenatal diagnosis
Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration.1.2We report on a neonate with Wilms tumor in a prenatally diagnosed multicystic dysplastic kidney. To our knowledge this is the fifth case of Wilms tumor arising in a multicystic dysplastic kidney but only the second diagnosed prenatally. CASE REPORT
A male neonate underwent physical examination, which revealed a left flank mass. Prenatal ultrasound had shown only multiple renal cysts, and a normal kidney on the left and right sides, respectively (see figure). Postnatal ultrasound confirmed renal cystic disease without visualization of the parenchyma and ureter. Voiding cystourethrography was normal. Dimemapto-succinic acid scan at age 2 months demonstrated complete absence of function on the left side and normal function on the right side. The diagnosis was multicystic dysplastic kidney. A conservative approach involved ultrasound followup every 6 months. At age 14 months ultrasound done elsewhere showed persistent left renal cystic disease with no evidence of tumor. Four months later the mother noticed increased abdominal girth and enlargement of the left flank mass. Ultrasound and computerized tomography revealed a 16 X 10.5 X 13 cm. solid tumor in the left renal fossa with calcifications. Total left nephrectomy and lymph node sampling were performed via upper abdominal transverse laparotomy. The ureter was atretic, the contralateral kidney was normal and no tumor spillage occurred. Convalescence was uneventful and the patient was discharged from the hospital on day 3. Pathological examination revealed favorable histology Wilms tumor with invasion of the perirenal fat. There were areas of necrosis within the tumor but no significant bleeding to explain the sudden increase in tumor size. All lymph nodes were free of tumor. The patient was enrolled in the stage I1 arm of the Brazilian Group for Treatment of Wilms Tumor. He received vincristine and dactinomycin chemotherapy for 6 months, and is now in good health and free of disease 13 months after therapy. DISCUSSION
Multicystic dysplastic kidney is a form of cystic renal disease in which the renal parenchyma is replaced by tense noncommunicating cysts associated with a nonpatent drainage system. This condition is common and it can be detected on prenatal ultrasound. Complications related to multicystic dysplastic kidney are rare but infection, hypertension, abdominal pain and malignant degeneration have been described.’.‘ Wacksman and Phipps reported that 260 of the 441 children enrolled in the Multicystic Kidney Registry Accepted for publication April 4, 1997.
Prenatal ultrasound of left kidney shows multiple cysts
were treated nonoperatively without complications. In those who underwent surgery nephrectomy was performed for reasons other than hypertension, infection, pain or tumor. They also observed that multicystic dysplastic kidney may take as long as 20 years to regress and, therefore, long-term followup is necessary. In accordance with these findings others have advocated a nonsurgical approach to multicystic dysplastic kidney. The main reason for nonsurgical therapy is a lack of evidence that any of these kidneys had progression to neoplasia. However, 10 cases of malignancy have been reported of which 5, including ours, were identified as Wilms tumor.2 Although the multicystic dysplastic kidney image disappears on ultrasound, dysplastic tissue may remain and it can be identified histologically.2 Corsi et a1 reported a case of multicystic dysplastic kidney with a nodular renal blastema, a form of nephroblastematosis, which is considered a precursor to and the first morphological expression of the Knudson 2-hit theory in the genesis of Wilms tumor.3 Based on a literature review the risk of complications in multicystic dysplastic kidney is low and nonoperative management is the current recommended approach. However, due to the extreme difficulty of close, long, active followup with ultrasound and physical examination, and the feasibility of nephrectomy done on an outpatient basis with minimal morbidity, we recommend that the role of surgical management be reconsidered and prompt surgical intervention be performed at the earliest suspicious change.
Dr. Anthony Khouri, Hospital for Sick Children, Toronto, Canada provided critical advice. 1926
WILMS TUMOR IN PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY REFERENCES
1. Wacksman, J. and Phipps, L.: Report of the multicystic kidney registry: preliminary findings. J. Urol., 150: 1870, 1993. 2. Homsy, Y. L., Anderson, J. H., Oudjhane, K. and Russo, P.: Wilms’ tumor in multicystic dysplastic kidney disease. J. Urol., in press. 3. Corsi, A.,Boldrini, R., Caione, P. and Bosman, C.: Nodular renal blastoma in kidney with multicystic dysplasia. Report of a case. Pathologica, 87: 183,1995.
EDITORIAL COMMENT The authors are to be congratulated on following through and identifymg Wilms tumor in a prenatally diagnosed multicystic kidney. As pointed out by Beckwith in previous communications on multicystic kidneys, true multicystic kidneys are not at increased risk for Wilms tumors. In his experience with more than 8,500 pediatric renal tumors only 3 cases of Wilms tumor arising in a dysplastic kidney were encountered. All of these cases have been fled in the National Wilms Tumor Study. Because of his data and those of the Multicystic Kidney Registry, we believe that these patients should be followed but we should continue to question the need for nephrectomy in all with multicystic kidney disease. It has been pointed out that these patients should be followed closely every 3 to 6 months in year 1of life. Ultrasound should also be reviewed by the
1927
urologist or surgeon with the radiologist to ensure that a multicystic kidney has been correctly diagnosed. In addition, the fact that the prenatally diagnosed disorder was the same disease postnatally has not been a uniform experience. There have been many cases of multicystic kidneys diagnosed prenatally in which postnatal evaluation has not confirmed the prenatal diagnosis. In our experience we hesitate to reassure parents that the child indeed has a multicystic kidney, since there are other renal anomalies that may masquerade as a multicystic kidney, such as hydmnephrosis and duplication anomalies with cystic dysplasia. Again, the authors should be congratulated for bringing this report to our attention. We urge others to register any multicystic kidneys with the Multicystic Kidney kgistry, so that they may be followed prospectively. Jeffrs, Wacksman Pediatric Urology Division Children’s Hospital Medical Center Cincinnati, Ohio REPLY BY AUTHORS We wish to emphasize that, although rare,a malignant degeneration in a mrreetly diagnosed multicystic kidney may occw and lifetime followup is neceymy. When long-term followup is ditticult due to parental andor d problems a surgical therapy should be amsidered.
WILMS TUMOR IN A PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY ANTONIO GONCALVES DE OLIVEIRA-FILHO, MARfLIA HOJAIJ CAFWALHO, LOURENCO SBRAGIA-NETO, M h C I O LOPES MIRANDA, JOAQUIM MURRAY BUSTORFF-SILVA AND EDISON RISSATO DE OLIVEIRA From the Departments of Surgery and Anesthesiology, Boldrini’s Children Cancer Center and Department of Pediatric Surgery, University of Campinas, Campinas, Brazil
KEYWORDS:kidney, polycystie;Wilms’ tumor; prenatal diagnosis
Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration.1.2We report on a neonate with Wilms tumor in a prenatally diagnosed multicystic dysplastic kidney. To our knowledge this is the fifth case of Wilms tumor arising in a multicystic dysplastic kidney but only the second diagnosed prenatally. CASE REPORT
A male neonate underwent physical examination, which revealed a left flank mass. Prenatal ultrasound had shown only multiple renal cysts, and a normal kidney on the left and right sides, respectively (see figure). Postnatal ultrasound confirmed renal cystic disease without visualization of the parenchyma and ureter. Voiding cystourethrography was normal. Dimemapto-succinic acid scan at age 2 months demonstrated complete absence of function on the left side and normal function on the right side. The diagnosis was multicystic dysplastic kidney. A conservative approach involved ultrasound followup every 6 months. At age 14 months ultrasound done elsewhere showed persistent left renal cystic disease with no evidence of tumor. Four months later the mother noticed increased abdominal girth and enlargement of the left flank mass. Ultrasound and computerized tomography revealed a 16 X 10.5 X 13 cm. solid tumor in the left renal fossa with calcifications. Total left nephrectomy and lymph node sampling were performed via upper abdominal transverse laparotomy. The ureter was atretic, the contralateral kidney was normal and no tumor spillage occurred. Convalescence was uneventful and the patient was discharged from the hospital on day 3. Pathological examination revealed favorable histology Wilms tumor with invasion of the perirenal fat. There were areas of necrosis within the tumor but no significant bleeding to explain the sudden increase in tumor size. All lymph nodes were free of tumor. The patient was enrolled in the stage I1 arm of the Brazilian Group for Treatment of Wilms Tumor. He received vincristine and dactinomycin chemotherapy for 6 months, and is now in good health and free of disease 13 months after therapy. DISCUSSION
Multicystic dysplastic kidney is a form of cystic renal disease in which the renal parenchyma is replaced by tense noncommunicating cysts associated with a nonpatent drainage system. This condition is common and it can be detected on prenatal ultrasound. Complications related to multicystic dysplastic kidney are rare but infection, hypertension, abdominal pain and malignant degeneration have been described.’.‘ Wacksman and Phipps reported that 260 of the 441 children enrolled in the Multicystic Kidney Registry Accepted for publication April 4, 1997.
Prenatal ultrasound of left kidney shows multiple cysts
were treated nonoperatively without complications. In those who underwent surgery nephrectomy was performed for reasons other than hypertension, infection, pain or tumor. They also observed that multicystic dysplastic kidney may take as long as 20 years to regress and, therefore, long-term followup is necessary. In accordance with these findings others have advocated a nonsurgical approach to multicystic dysplastic kidney. The main reason for nonsurgical therapy is a lack of evidence that any of these kidneys had progression to neoplasia. However, 10 cases of malignancy have been reported of which 5, including ours, were identified as Wilms tumor.2 Although the multicystic dysplastic kidney image disappears on ultrasound, dysplastic tissue may remain and it can be identified histologically.2 Corsi et a1 reported a case of multicystic dysplastic kidney with a nodular renal blastema, a form of nephroblastematosis, which is considered a precursor to and the first morphological expression of the Knudson 2-hit theory in the genesis of Wilms tumor.3 Based on a literature review the risk of complications in multicystic dysplastic kidney is low and nonoperative management is the current recommended approach. However, due to the extreme difficulty of close, long, active followup with ultrasound and physical examination, and the feasibility of nephrectomy done on an outpatient basis with minimal morbidity, we recommend that the role of surgical management be reconsidered and prompt surgical intervention be performed at the earliest suspicious change.
Dr. Anthony Khouri, Hospital for Sick Children, Toronto, Canada provided critical advice. 1926
WILMS TUMOR IN PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY REFERENCES
1. Wacksman, J. and Phipps, L.: Report of the multicystic kidney registry: preliminary findings. J. Urol., 150: 1870, 1993. 2. Homsy, Y. L., Anderson, J. H., Oudjhane, K. and Russo, P.: Wilms’ tumor in multicystic dysplastic kidney disease. J. Urol., in press. 3. Corsi, A.,Boldrini, R., Caione, P. and Bosman, C.: Nodular renal blastoma in kidney with multicystic dysplasia. Report of a case. Pathologica, 87: 183,1995.
EDITORIAL COMMENT The authors are to be congratulated on following through and identifymg Wilms tumor in a prenatally diagnosed multicystic kidney. As pointed out by Beckwith in previous communications on multicystic kidneys, true multicystic kidneys are not at increased risk for Wilms tumors. In his experience with more than 8,500 pediatric renal tumors only 3 cases of Wilms tumor arising in a dysplastic kidney were encountered. All of these cases have been fled in the National Wilms Tumor Study. Because of his data and those of the Multicystic Kidney Registry, we believe that these patients should be followed but we should continue to question the need for nephrectomy in all with multicystic kidney disease. It has been pointed out that these patients should be followed closely every 3 to 6 months in year 1of life. Ultrasound should also be reviewed by the
1927
urologist or surgeon with the radiologist to ensure that a multicystic kidney has been correctly diagnosed. In addition, the fact that the prenatally diagnosed disorder was the same disease postnatally has not been a uniform experience. There have been many cases of multicystic kidneys diagnosed prenatally in which postnatal evaluation has not confirmed the prenatal diagnosis. In our experience we hesitate to reassure parents that the child indeed has a multicystic kidney, since there are other renal anomalies that may masquerade as a multicystic kidney, such as hydmnephrosis and duplication anomalies with cystic dysplasia. Again, the authors should be congratulated for bringing this report to our attention. We urge others to register any multicystic kidneys with the Multicystic Kidney kgistry, so that they may be followed prospectively. Jeffrs, Wacksman Pediatric Urology Division Children’s Hospital Medical Center Cincinnati, Ohio REPLY BY AUTHORS We wish to emphasize that, although rare,a malignant degeneration in a mrreetly diagnosed multicystic kidney may occw and lifetime followup is neceymy. When long-term followup is ditticult due to parental andor d problems a surgical therapy should be amsidered.