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MULTIMODAL THERAPY FOR STAGE IV ADULT WILMS TUMOR
0022-5347/01/1654-1202/0 THE JOURNAL OF UROLOGY® Copyright © 2001 by AMERICAN UROLOGICAL ASSOCIATION, INC.®
Vol. 165, 1202, April 2001 Printed in U.S.A.
MULTIMODAL THERAPY FOR STAGE IV ADULT WILMS TUMOR GANESH V. RAJ, SCOTT K. PRUITT, SCOTT LILLY, JOHN T. MADDEN
AND
DAVID T. PRICE
From the Division of Urology, Departments of Surgery, Medicine and Pathology, Duke University Medical Center, Durham, North Carolina KEY WORDS: nephroblastoma; drug therapy, combination; kidney; neoplasm metastasis; liver
Wilms tumor or nephroblastoma is the most common abdominal malignancy in children. It is rare in adults and associated with a worse prognosis due to higher tumor stages at presentation, more frequent recurrence and poor response to chemotherapy. Optimal treatment of advanced stage Wilms tumor in adults has not been established. CASE REPORT
A 34-year-old Native American, with no significant medical history, presented with a 2 to 3 month history of persistent right low back pain, 10 lb. weight loss, and acute nausea and vomiting during a 2-week period. Abdominal ultrasonography revealed a large, predominantly solid, heterogeneous mass near the lower pole of an enlarged right kidney. Computerized tomography (CT) revealed a 15 cm. mass arising from the superior pole of the right kidney, multiple lesions in the liver and both lungs, with significant pleural effusions (fig. 1, A). Positron emission tomography verified fluorodeoxyglucose uptake by these lesions in the liver and lungs, consistent with metastatic lesions. Additional staging included a bone scan, brain magnetic resonance imaging, testicular ultrasound, tumor markers and urinalysis, which were all normal. CT guided core biopsy of the renal mass was performed. Histopathological analysis revealed malignant small blue cells, consistent with Wilms tumor of a favorable histology (fig. 2). Pathological diagnosis was reviewed and independently confirmed by the director of the National Wilms’ Tumor Study pathology section. The patient was treated preoperatively according to a 48week pediatric high risk Wilms tumor protocol, which consisted of 5 cycles of multiagent chemotherapy (cyclophosphamide, etoposide, doxorubicin, vincristine and dactinomycin). Right radical nephrectomy was performed with the placement of a right hepatic artery perfusion catheter (Arrow Accepted for publication November 17, 2000.
FIG. 2. Island of blastema with interspersed tubules typical of nephroblastoma. Note the absence of anaplasia. H & E, reduced from ⫻325.
pump*). Mediastinum and lungs were subjected to 2,000 cGy. radiation. A month after resection, systemic chemotherapy with vincristine, dactinomycin, cyclophosphamide and etoposide, and intrahepatic therapy (via the Arrow pump) with doxorubicin was resumed. Subsequently, the patient received 2 cycles of continuous intrahepatic infusion of doxorubicin, and a course of systemic 200 mg./m.2 paclitaxel. Followup CT at 6 months after resection showed resolution of lung metastases, stable liver lesions and no residual disease in the renal bed (fig. 1, B). DISCUSSION
Most reports of advanced cases of adult Wilms tumor with metastases have not been favorable1 and have indicated the superiority of multimodale therapy over singular approaches.2 Most adults with stage IV Wilms tumor responded poorly to actinomycin, vincristine, doxorubicin, cisplatin or etoposide based regimens.1, 3 We report the possible efficacy of an aggressive combined regimen of preoperative and postoperative chemotherapy with surgery and radiation to treat a case of adult Wilms tumor. A 5-drug chemotherapeutic regimen was used with monthly CT to monitor the efficacy of the therapy. We believe that the use of intrahepatic chemotherapy allows for better management of hepatic metastasis. At 12-month followup the tumor appeared to be under control. Further evaluation of this approach with additional patients will help us evaluate its success. REFERENCES
1. Bozeman, G., Bissada, N. K., Abboud, M. R. et al: Adult Wilms’ tumor: prognosis and management considerations. Urology, 45: 1055, 1995 2. Dawson, N. A., Klein, M. A. and Taylor, H. G.: Salvage therapy in metastatic adult Wilms’ tumor. Cancer, 62: 1017, 1988 3. Kattan, J., Tournade, M. F., Culine, S. et al: Adult Wilms’ tumour: review of 22 cases. Eur J Cancer, 30A: 1778, 1994 FIG. 1. Abdominal CT shows hepatic mass before (A) and after (B) combined therapy. * Arrow International Inc., Wampole, Massachusetts. 1202
Vol. 165, 1202, April 2001 Printed in U.S.A.
MULTIMODAL THERAPY FOR STAGE IV ADULT WILMS TUMOR GANESH V. RAJ, SCOTT K. PRUITT, SCOTT LILLY, JOHN T. MADDEN
AND
DAVID T. PRICE
From the Division of Urology, Departments of Surgery, Medicine and Pathology, Duke University Medical Center, Durham, North Carolina KEY WORDS: nephroblastoma; drug therapy, combination; kidney; neoplasm metastasis; liver
Wilms tumor or nephroblastoma is the most common abdominal malignancy in children. It is rare in adults and associated with a worse prognosis due to higher tumor stages at presentation, more frequent recurrence and poor response to chemotherapy. Optimal treatment of advanced stage Wilms tumor in adults has not been established. CASE REPORT
A 34-year-old Native American, with no significant medical history, presented with a 2 to 3 month history of persistent right low back pain, 10 lb. weight loss, and acute nausea and vomiting during a 2-week period. Abdominal ultrasonography revealed a large, predominantly solid, heterogeneous mass near the lower pole of an enlarged right kidney. Computerized tomography (CT) revealed a 15 cm. mass arising from the superior pole of the right kidney, multiple lesions in the liver and both lungs, with significant pleural effusions (fig. 1, A). Positron emission tomography verified fluorodeoxyglucose uptake by these lesions in the liver and lungs, consistent with metastatic lesions. Additional staging included a bone scan, brain magnetic resonance imaging, testicular ultrasound, tumor markers and urinalysis, which were all normal. CT guided core biopsy of the renal mass was performed. Histopathological analysis revealed malignant small blue cells, consistent with Wilms tumor of a favorable histology (fig. 2). Pathological diagnosis was reviewed and independently confirmed by the director of the National Wilms’ Tumor Study pathology section. The patient was treated preoperatively according to a 48week pediatric high risk Wilms tumor protocol, which consisted of 5 cycles of multiagent chemotherapy (cyclophosphamide, etoposide, doxorubicin, vincristine and dactinomycin). Right radical nephrectomy was performed with the placement of a right hepatic artery perfusion catheter (Arrow Accepted for publication November 17, 2000.
FIG. 2. Island of blastema with interspersed tubules typical of nephroblastoma. Note the absence of anaplasia. H & E, reduced from ⫻325.
pump*). Mediastinum and lungs were subjected to 2,000 cGy. radiation. A month after resection, systemic chemotherapy with vincristine, dactinomycin, cyclophosphamide and etoposide, and intrahepatic therapy (via the Arrow pump) with doxorubicin was resumed. Subsequently, the patient received 2 cycles of continuous intrahepatic infusion of doxorubicin, and a course of systemic 200 mg./m.2 paclitaxel. Followup CT at 6 months after resection showed resolution of lung metastases, stable liver lesions and no residual disease in the renal bed (fig. 1, B). DISCUSSION
Most reports of advanced cases of adult Wilms tumor with metastases have not been favorable1 and have indicated the superiority of multimodale therapy over singular approaches.2 Most adults with stage IV Wilms tumor responded poorly to actinomycin, vincristine, doxorubicin, cisplatin or etoposide based regimens.1, 3 We report the possible efficacy of an aggressive combined regimen of preoperative and postoperative chemotherapy with surgery and radiation to treat a case of adult Wilms tumor. A 5-drug chemotherapeutic regimen was used with monthly CT to monitor the efficacy of the therapy. We believe that the use of intrahepatic chemotherapy allows for better management of hepatic metastasis. At 12-month followup the tumor appeared to be under control. Further evaluation of this approach with additional patients will help us evaluate its success. REFERENCES
1. Bozeman, G., Bissada, N. K., Abboud, M. R. et al: Adult Wilms’ tumor: prognosis and management considerations. Urology, 45: 1055, 1995 2. Dawson, N. A., Klein, M. A. and Taylor, H. G.: Salvage therapy in metastatic adult Wilms’ tumor. Cancer, 62: 1017, 1988 3. Kattan, J., Tournade, M. F., Culine, S. et al: Adult Wilms’ tumour: review of 22 cases. Eur J Cancer, 30A: 1778, 1994 FIG. 1. Abdominal CT shows hepatic mass before (A) and after (B) combined therapy. * Arrow International Inc., Wampole, Massachusetts. 1202