Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy

J Pediatr Surg. 2011 Dec;46(12):e1-6. doi: 10.1016/j.jpedsurg.2011.09.049.

Abstract

Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Fewer than 30 cases have been described. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. The patient continued to survive despite cessation of treatment. The overall predominance of differentiated stromal elements in this subtype of Wilms' tumor might explain the poor response to chemotherapy yet generally favorable outcome. Recognition of this subtype on biopsy might justify earlier surgical intervention that, for bilateral tumors, might allow for greater nephron sparing.

Publication types

  • Case Reports

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Carboplatin / administration & dosage
  • Dactinomycin / administration & dosage
  • Disease Progression
  • Doxorubicin / administration & dosage
  • Drug Resistance, Neoplasm
  • Etoposide / administration & dosage
  • Humans
  • Ifosfamide / administration & dosage
  • Infant
  • Kidney Neoplasms / classification
  • Kidney Neoplasms / drug therapy
  • Kidney Neoplasms / pathology*
  • Kidney Neoplasms / surgery
  • Male
  • Neoadjuvant Therapy
  • Neoplasms, Multiple Primary / drug therapy
  • Neoplasms, Multiple Primary / pathology*
  • Neoplasms, Multiple Primary / surgery
  • Nephrectomy
  • Prognosis
  • Stromal Cells / pathology
  • Vincristine / administration & dosage
  • Wilms Tumor / classification
  • Wilms Tumor / drug therapy
  • Wilms Tumor / pathology*
  • Wilms Tumor / surgery

Substances

  • Dactinomycin
  • Vincristine
  • Etoposide
  • Doxorubicin
  • Carboplatin
  • Ifosfamide