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Teratoid Wilms' tumor: the St Jude experience
E T Fernandes1, D M Parham, R C Ribeiro
1Division of Surgery, St Jude Children's Research Hospital, Memphis, TN 38101.
Journal of Pediatric Surgery
|December 1, 1988
Summary
Teratoid Wilms' tumors, rare kidney cancers with diverse cell types, show poor response to standard treatments. Surgery is the primary therapy due to resistance to chemotherapy and irradiation.
Area of Science:
- Pediatric Oncology
- Nephropathology
Background:
- Wilms' tumor is a common pediatric kidney cancer.
- Teratoid Wilms' tumor is a rare subtype characterized by diverse cell types alongside classic nephroblastoma tissue.
Observation:
- Three cases of teratoid Wilms' tumor were identified among 290 patients treated between 1964 and 1987.
- Two patients presented with bilateral pyeloureteral obstruction, uremia, and hypertension.
Findings:
- Teratoid Wilms' tumors showed poor response to chemotherapy and irradiation compared to classic nephroblastoma.
- One child died from sepsis and renal failure; two are surviving disease-free.
Implications:
- The unique presentation and treatment resistance highlight the need for specialized management.
- Surgery is recommended as the principal therapeutic approach for teratoid Wilms' tumors.