0022-5347/78/1203-0354$02.00/0 THE JOURNAL OF UROLOGY Copyright © 1978 by The Williams & Wilkins Co.
Vol. 120, September Printed in U.S.A.
CONGENITAL HYDRONEPHROSIS WITH IPSILATERAL ADRENAL NEUROBLASTOMA: A CASE REPORT N. BANSAL, M. WAINSTEIN
AND
K. A. KROPP*
From the Toledo Hospital and Medical College of Ohio at Toledo, Toledo, Ohio
ABSTRACT
A case ofright flank mass owing to congenital hydronephrosis and coexisting with an ipsilateral adrenal neuroblastoma in a 21-day-old female newborn is reported. subcostal incision was done. When the peritoneum was opened there was no ascites. The liver contained multiple small, yellowish nodules. A distinct mass could be palpated in the right suprarenal area in addition to the hydronephrotic renal pelvis. There were multiple palpable lymph nodes along the inferior vena cava. A frozen section of lymph node was compatible with a neuroblastoma. A right radical nephrectomy and adrenalectomy with retroperitoneal lymphadenec-
Neuroblastoma may have varied manifestations. One of the characteristics of a neuroblastoma is the vagueness of symptomatology that often leads to delay in diagnosis. Less than half of the children may present with a palpable abdominal mass. 1 Herein we present an unusual case of a right flank mass owing to congenital hydronephrosis with ureteropelvic junction obstruction and an ipsilateral adrenal neuroblastoma discovered at operation.
Fm. 1. A, IVP with nephrotomogram (15 minutes) shows inferolateral displacement of right renal pelvis and normal left pyelocaliceal system. B, delayed (6-hour) film reveals large hydronephrotic extrarenal pelvis. CASE REPORT
J. D., 10634, a 21-day-old female newborn, was hospitalized on July 25, 1975 with a soft cystic abdominal mass in the right upper quadrant and flank discovered on routine physical examination by the pediatrician. Hemoglobin was 12.5, hematocrit 37, white blood count 12.6, blood urea nitrogen 8, creatinine 0.8, urinalysis and culture and sensitivity negative, and bilirubin 1.4. The chest x-ray was normal. An excretory urogram (IVP) showed hydronephrosis on the right side with a large extrarenal pelvis. The upper calix was not well defined and the kidney appeared to be displaced slightly downward and laterally (fig. 1). There was no evidence of calcification in the suprarenal area. A voiding cystourethrogram showed no reflux. Cystoscopy was normal and showed no evidence ofureteral duplication. A right retrograde pyelogram revealed a large hydronephrotic, extrarenal pelvis probably owing to ureteropelvic junction obstruction (fig. 2). A transabdominal exploration through a right anterior Accepted for publication January 27, 1978. *Requests for reprints: Department of Urology, Medical College of Ohio at Toledo, C. S. No. 10008, Toledo, Ohio 43699.
tomy were accomplished with difficulty. A wedge of liver also was excised for biopsy. Convalescence was uneventful. The final pathology report showed a hydronephrosis with intrinsic stricture of the ureteropelvic junction (fig. 3). The right adrenal gland was replaced totally by the neuroblastoma with foci of calcification and maturation (fig. 4). The lymph nodes and liver contained metastatic neuroblastoma. Bone survey revealed no metastasis. Iliac crest marrow aspirate showed 5 to 8 isolated tumor cells per slide. Urinary vanillyl mandelic acid levels were 70 µ,g./mg. creatinine (normal range 0.6 to 12 µ,g./mg. creatinine). 2 Carcinoembryonic antigen levels were 2. 7 µ,g./ml. The tumor was classified initially as stage IV-s.:i The patient was given 1,800 rads to the right upper quadrant and para-aortic nodes immediately postoperatively. Chemotherapy was started with vincristine and cyclophosphamide. In November 1975 the urinary vanillyl mandelic acid increased to 176 µ,g./mg. creatinine. In December bilateral proptosis and eyelid edema developed. A bone scan showed increased uptake over the orbital area. On continued treatment with cyclophosphamide and vincristine intravenously the proptosis subsided gradually. The patient has done well and remains free of any
354
355
FIG. 2. A, right retrograde pyelogram with ureteral catheter in place. B, pull out film shows high insertion of ureteropelvic junction
FIG. 3. A, gross specimen shows large extrarenal hydronephrotic pelvis with high insertion ofureteropelvicjuncti_on and well preserved renal parenchyma. B, cut section of right adrenal gland reveals complete replacement of adrenal by tumor which is well confined
FIG. 4. Photomicrograph shows typical changes of neuroblastoma
residual disease with negative bone scan and bone marrow smear. DISCUSSION
Neuroblastoma is one of the most common childhood tumors and accounts for 7 per cent of cancer deaths in children less than 15 years old:1 In most series it ranks behind leukemia, lymphoma and central nervous system tumors but it is more common than Wilms tumor or rhabdomyosarcoma. Approximately a third of neuroblastomas present within the first year of life and have a better prognosis than at an older age. The location of the primary tumor, the extent of disease and histologic maturity of the neoplasm also affect the survival. The typical urographic feature in a case of neuroblastoma is angular displacement of the kidney without caliceal distortion. Occasionally, the tumor may invade the renal substance, causing caliceal deformity. Acute angulation of the ureteropelvic junction or pyelocalicectasis may be present in an extraadrenal neuroblastoma. Stippled calcification in suprarenal
areas may be present on plain film. Measurements of catecholamines and their metabolites are diagnostic in a majority of cases. Frens and associates reported elevated carcinoembryonic antigen levels in a neuroblastoma and indicated a more favorable outcome in cases with initial carcinoembryonic antigen levels less than 4.0 ng./mL" Our case is an unusual combination of congenital hydronephrosis presenting as an abdominal mass and ipsilateral adrenal neuroblastoma diagnosed incidentally at the time of surgical exploration. Because of slightly downward displacement of the right kidney, the nature of which was obscure preoperatively, a transabdominal approach was used. No attempt was made to conserve the kidney by doing a pyeloplasty because of danger of an anastomotic leak in the event of postoperative irradiation. Although the presence of a preoperatively undiagnosed neuroblastoma influenced our choice of surgical treatment of hydronephrosis the ultimate treatment and outcome of operation for the neuroblastoma have not been altered in any way. In fact, the presence of the hydroneresulted in an diagnosis of neuroblastorna. A combination of postoperative irradiation and chemotherapy has resulted in more than a survival free of disease in this particular case. REFERENCES
1. Donohue, J.P., Garrett, R. A., Baehner, R. L. and Thomas, M.
2. 3. 4. 5.
H.: The multiple manifestations of neuroblastoma. J. Urol., 111: 260, 1974. Williams, C. M. and Greer, M.: Homovanillic acid and vanilmandelic acid in diagnosis of neuroblastoma. J.A.M.A., 183: 836, 1963. Evans, A. E., D'Angio, G. J. and Randolph, J.: A proposed staging for children with neuroblastoma. Children's cancer study group A. Cancer, 27: 374, 1971. Miller, R. W.: Fifty-two forms of childhood cancer: United States mortality experience, 1960-1966. J. Pediat., 75: 685, 1969. Frens, D. M., Bray, F., Wu, J. T. and Lang, G. C.: The carcinoembryonic antigen (CEA) assay: prognostic implications in neural crest tumors (abstract). Pediat. Res., 9: 387, 1975.
Vol. 120, September Printed in U.S.A.
CONGENITAL HYDRONEPHROSIS WITH IPSILATERAL ADRENAL NEUROBLASTOMA: A CASE REPORT N. BANSAL, M. WAINSTEIN
AND
K. A. KROPP*
From the Toledo Hospital and Medical College of Ohio at Toledo, Toledo, Ohio
ABSTRACT
A case ofright flank mass owing to congenital hydronephrosis and coexisting with an ipsilateral adrenal neuroblastoma in a 21-day-old female newborn is reported. subcostal incision was done. When the peritoneum was opened there was no ascites. The liver contained multiple small, yellowish nodules. A distinct mass could be palpated in the right suprarenal area in addition to the hydronephrotic renal pelvis. There were multiple palpable lymph nodes along the inferior vena cava. A frozen section of lymph node was compatible with a neuroblastoma. A right radical nephrectomy and adrenalectomy with retroperitoneal lymphadenec-
Neuroblastoma may have varied manifestations. One of the characteristics of a neuroblastoma is the vagueness of symptomatology that often leads to delay in diagnosis. Less than half of the children may present with a palpable abdominal mass. 1 Herein we present an unusual case of a right flank mass owing to congenital hydronephrosis with ureteropelvic junction obstruction and an ipsilateral adrenal neuroblastoma discovered at operation.
Fm. 1. A, IVP with nephrotomogram (15 minutes) shows inferolateral displacement of right renal pelvis and normal left pyelocaliceal system. B, delayed (6-hour) film reveals large hydronephrotic extrarenal pelvis. CASE REPORT
J. D., 10634, a 21-day-old female newborn, was hospitalized on July 25, 1975 with a soft cystic abdominal mass in the right upper quadrant and flank discovered on routine physical examination by the pediatrician. Hemoglobin was 12.5, hematocrit 37, white blood count 12.6, blood urea nitrogen 8, creatinine 0.8, urinalysis and culture and sensitivity negative, and bilirubin 1.4. The chest x-ray was normal. An excretory urogram (IVP) showed hydronephrosis on the right side with a large extrarenal pelvis. The upper calix was not well defined and the kidney appeared to be displaced slightly downward and laterally (fig. 1). There was no evidence of calcification in the suprarenal area. A voiding cystourethrogram showed no reflux. Cystoscopy was normal and showed no evidence ofureteral duplication. A right retrograde pyelogram revealed a large hydronephrotic, extrarenal pelvis probably owing to ureteropelvic junction obstruction (fig. 2). A transabdominal exploration through a right anterior Accepted for publication January 27, 1978. *Requests for reprints: Department of Urology, Medical College of Ohio at Toledo, C. S. No. 10008, Toledo, Ohio 43699.
tomy were accomplished with difficulty. A wedge of liver also was excised for biopsy. Convalescence was uneventful. The final pathology report showed a hydronephrosis with intrinsic stricture of the ureteropelvic junction (fig. 3). The right adrenal gland was replaced totally by the neuroblastoma with foci of calcification and maturation (fig. 4). The lymph nodes and liver contained metastatic neuroblastoma. Bone survey revealed no metastasis. Iliac crest marrow aspirate showed 5 to 8 isolated tumor cells per slide. Urinary vanillyl mandelic acid levels were 70 µ,g./mg. creatinine (normal range 0.6 to 12 µ,g./mg. creatinine). 2 Carcinoembryonic antigen levels were 2. 7 µ,g./ml. The tumor was classified initially as stage IV-s.:i The patient was given 1,800 rads to the right upper quadrant and para-aortic nodes immediately postoperatively. Chemotherapy was started with vincristine and cyclophosphamide. In November 1975 the urinary vanillyl mandelic acid increased to 176 µ,g./mg. creatinine. In December bilateral proptosis and eyelid edema developed. A bone scan showed increased uptake over the orbital area. On continued treatment with cyclophosphamide and vincristine intravenously the proptosis subsided gradually. The patient has done well and remains free of any
354
355
FIG. 2. A, right retrograde pyelogram with ureteral catheter in place. B, pull out film shows high insertion of ureteropelvic junction
FIG. 3. A, gross specimen shows large extrarenal hydronephrotic pelvis with high insertion ofureteropelvicjuncti_on and well preserved renal parenchyma. B, cut section of right adrenal gland reveals complete replacement of adrenal by tumor which is well confined
FIG. 4. Photomicrograph shows typical changes of neuroblastoma
residual disease with negative bone scan and bone marrow smear. DISCUSSION
Neuroblastoma is one of the most common childhood tumors and accounts for 7 per cent of cancer deaths in children less than 15 years old:1 In most series it ranks behind leukemia, lymphoma and central nervous system tumors but it is more common than Wilms tumor or rhabdomyosarcoma. Approximately a third of neuroblastomas present within the first year of life and have a better prognosis than at an older age. The location of the primary tumor, the extent of disease and histologic maturity of the neoplasm also affect the survival. The typical urographic feature in a case of neuroblastoma is angular displacement of the kidney without caliceal distortion. Occasionally, the tumor may invade the renal substance, causing caliceal deformity. Acute angulation of the ureteropelvic junction or pyelocalicectasis may be present in an extraadrenal neuroblastoma. Stippled calcification in suprarenal
areas may be present on plain film. Measurements of catecholamines and their metabolites are diagnostic in a majority of cases. Frens and associates reported elevated carcinoembryonic antigen levels in a neuroblastoma and indicated a more favorable outcome in cases with initial carcinoembryonic antigen levels less than 4.0 ng./mL" Our case is an unusual combination of congenital hydronephrosis presenting as an abdominal mass and ipsilateral adrenal neuroblastoma diagnosed incidentally at the time of surgical exploration. Because of slightly downward displacement of the right kidney, the nature of which was obscure preoperatively, a transabdominal approach was used. No attempt was made to conserve the kidney by doing a pyeloplasty because of danger of an anastomotic leak in the event of postoperative irradiation. Although the presence of a preoperatively undiagnosed neuroblastoma influenced our choice of surgical treatment of hydronephrosis the ultimate treatment and outcome of operation for the neuroblastoma have not been altered in any way. In fact, the presence of the hydroneresulted in an diagnosis of neuroblastorna. A combination of postoperative irradiation and chemotherapy has resulted in more than a survival free of disease in this particular case. REFERENCES
1. Donohue, J.P., Garrett, R. A., Baehner, R. L. and Thomas, M.
2. 3. 4. 5.
H.: The multiple manifestations of neuroblastoma. J. Urol., 111: 260, 1974. Williams, C. M. and Greer, M.: Homovanillic acid and vanilmandelic acid in diagnosis of neuroblastoma. J.A.M.A., 183: 836, 1963. Evans, A. E., D'Angio, G. J. and Randolph, J.: A proposed staging for children with neuroblastoma. Children's cancer study group A. Cancer, 27: 374, 1971. Miller, R. W.: Fifty-two forms of childhood cancer: United States mortality experience, 1960-1966. J. Pediat., 75: 685, 1969. Frens, D. M., Bray, F., Wu, J. T. and Lang, G. C.: The carcinoembryonic antigen (CEA) assay: prognostic implications in neural crest tumors (abstract). Pediat. Res., 9: 387, 1975.
