KIDNEY TUMORS: NEPHROBLASTOMA/WILMS TUMOR

Nephroblastoma is the most common kidney tumor affecting young patients with about 550 new cases diagnosed annually in the United States. The therapy has evolved considerably during the past 30 years and the current excellent outcome in terms of both increased survival and reduced morbidity can be attributed to the randomized clinical trials undertaken since 1969. The treatment has been modified to include appropriate chemotherapy and limited radiation therapy to compliment the surgical removal of the tumor mass. Two-thirds of the patients have initial disease localized to the region of the kidney while almost 25% have more widespread disease within the abdomen and 10% show metastases to other parts of the body. Therapy can be specifically tailored for low risk patients which will reduce the morbidity of treatment and intensive therapy can be selected for those high-risk patients for whom survival remains poor.

In general, Wilms tumor is a disease of younger children affecting them at an average of three years of age. In approximately 95% of cases only one kidney is directly affected allowing for a targeted surgical approach which forms the cornerstone of therapy in most incidences. The surgeon has the responsibility to assess the extent of local tumor spread and perform safe and complete removal of the mass. Accurate staging is essential to determine the chemotherapy regimen and need for radiation therapy. Intraoperative events that can have an adverse impact on patient survival include tumor spill, incomplete removal and surgical complications. The other kidney, the lymph node glands in the region and the associated blood vessels are carefully evaluated. Tumor biopsies, preoperative rupture and surgical spillage are all factors, which may impact outcome. While the primary treatment is appropriate surgery there are several groups of patients in whom preoperative chemotherapy is recommended. These include children with both kidneys involved, those with exceptionally large tumors, or those with extensive involvement of the major blood vessels. These circumstances require assessment by pediatric oncologists and surgeons experienced in the treatment of this tumor.

With effective therapy, the overall outcome can be expected to be excellent. The four-year survival rate for patients with involvement localized to one kidney is between 90 and 95%. Even with regional tumors confined to the abdomen, 85 to 90% of children can expect to survive. Impressively, current multidisciplinary therapy in children with metastatic kidney tumors can demonstrate 80% survival if the inherent microscopic picture of the tumor does not show unfavorable aggressive features. The surgical guidelines for the safest tumor removal are established but the magnitude of the procedure may lead to complications. Bowel obstruction, hemorrhage, and injury to internal structures or major blood vessels do occur. More recently, it has been determined that intraoperative tumor spill may not only increase the rate of local regrowth of the tumor but also cause a higher mortality incidence if there was diffuse contamination. Surgical expertise in dealing with these childhood kidney tumors remains important.

SELECTED ARTICLES

  1. Ritchey ML, Shamberger RC, Haase GM, Horwitz J, Bergemann T, Breslow NE: Surgical Complications after Primary Nephrectomy for Wilms' Tumor: report from the National Wilms' Tumor Study Group. J Am Coll Surg 192:63-68, 2001.
  2. Shamberger RC, Ritchey ML, Haase GM, Bergemann TL, Loechelt-Yoshioka, Breslow NE, Green DM: Intravascular extension of Wilms' Tumor: Ann Surg 234: 116-121, 2001.
  3. Shamberger RC, Guthrie KA, Ritchey ML, Haase GM, Takashima J, Beckwith JB, D’Angio GJ, Green DM, Breslow NE. Surgery-related factors and local recurrence of Wilms tumor in National Wilms Tumor Study-4. Ann Surg 228:292-297, 1999.
  4. Haase GM, Ritchey ML. Nephroblastoma. Semin Pediatr Surg 6:11-16, 1997.
  5. Haase GM, Current surgical management of Wilms’ tumor. Curr Opin Pediatr 8:268-275, 1996.

 OTHER PUBLICATIONS OF INTEREST

  1. Neville H, Ritchey ML, Shamberger RC, Haase G, Perlman S, Yoshioka T: The Occurence of Wilms' Tumor in Horesehoe Kidneys: A Report from the National Wilms' Tumor Study Group (NWTSG). J Pediatr Surg 37: 1134-1137, 2002.

  2. Green DM, Breslow NE, Beckwith JB, Ritchey ML, Shamberger RC, Haase GM, D'Angio GJ, Perlman E, Donaldson M, Grundy PE, Weetman R, Coppes MJ, Malagolowkin M, Shearer P, Coccia P, Kletzel M, Thomas PRM, Macklis R, Tomlinson G, Huff V, Newbury R, Weeks D: Treatment with nephrectomy only for small stage I/favorable histology Wilms' tumor: A report from the National Wilms' Tumor Study Group. J Clin Oncol 19:3719-3724, 2001.

  3. Green DM, Breslow NE, Beckwith JB, Finklestein JZ, Grundy PE, Thomas PRM, Kim T, Shochat SJ, Haase GM, Ritchey ML, Kelalis PP, D’Angio GJ. Comparison between single dose and divided dose administration of dactinomycin and doxorubicin for patients with Wilms Tumor: A report from the National Wilms Tumor Study Group. J Clin Oncol 16:237-245, 1998.

  4. Horwitz JR, Ritchey ML, Moksness J, Breslow NE, Smith GR, Thomas PRM, Haase GM, Shamberger R, Beckwith JB: Renal salvage procedures in patients with synchronous bilateral Wilms’ tumors: a report from the National Wilms’ Tumor Study Group. J Pediatr Surg 31:1020-1025, 1996.
  5. 3. Ritchey ML, Azizkhan RG, Beckwith JB, Hrabovski EE, Haase GM: Neonatal Wilm’s tumor. J Pediatr Surg 30:856-859, 1995.
  6. Ritchey ML, Haase GM, Shochat SJ, Current management of Wilms’ tumor, Semin Surg Oncol 9:502-509, 1993.
  7. Ritchey ML, Kelalis PP, Haase GM, Shochat SJ: Preoperative chemotherapy for intracaval and atrial tumor extension of Wilms’ tumor. Cancer 71:4104-4110, 1993.
  8. Haase GM, Editorial: Controversies in Wilms’ Tumor, Dialogues in Pediatric Urology  edited by Koyle MA, William Miller and Assoc. Inc., Pearl River, NY 14 (12):7-8, 1991.
  9. Haase GM, Schoelkopf L, Beckwith JB: Classification, management and outcome in extrarenal Wilms’ tumor of childhood. Med Pediatr Oncol 19(5) 397-398, 1991.
  10. Arrigo S, Beckwith JB, Sharples K, D’Angio GJ, Haase GM: Adults with Wilms’ tumor (WT): Better survival after combined modality care. A report from the National Wilms’ Tumor Study (NWTS). Med Pediatr Oncol 18(5): 407, 1990.

     

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