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Recent experience with Wilms' tumor: 1978-1991
R C Shamberger1, R M Macklis, S E Sallan
1Department of Surgery, Children's Hospital, Boston, Massachusetts 02115.
Annals of Surgical Oncology
|January 1, 1994
Summary
Wilms tumor treatment has advanced, with current therapies tailored to stage and pathology. Some stage I Wilms tumors may be best observed without adjuvant therapy to minimize treatment toxicity.
Area of Science:
- Pediatric Oncology
- Nephrology
- Cancer Research
Background:
- Wilms tumor treatment has evolved significantly over the past four decades.
- Current therapeutic strategies are informed by tumor stage and histological subtype.
- This study assesses treatment outcomes from protocols designed to minimize doxorubicin use.
Purpose of the Study:
- To evaluate the efficacy of Wilms tumor treatment protocols.
- To assess outcomes in patients treated with regimens generally avoiding doxorubicin.
- To identify potential treatment de-escalation strategies for specific Wilms tumor subtypes.
Main Methods:
- Retrospective review of 114 children with renal tumors treated between 1978 and 1991.
- Classification of tumors into favorable histology Wilms tumor (91 patients) and unfavorable histology (23 patients).
- Chemotherapy regimens evolved over two eras (1978-1982 and 1983-1991), with doxorubicin reserved for specific cases or regimens.
- Radiotherapy was administered to the tumor bed in approximately two-thirds of patients.
Main Results:
- No isolated local failures were observed in favorable histology patients receiving radiotherapy.
- Event-free survival did not differ significantly among favorable histology patients across different stages.
- Patients with rhabdoid/sarcomatous Wilms tumor variants showed significantly poorer survival (p=0.00).
- Stage I tumors treated with nephrectomy alone demonstrated excellent outcomes (100% survival).
Conclusions:
- Wilms tumors remain highly curable, with survival rates exceeding expectations.
- Certain unfavorable histology Wilms tumors necessitate intensive therapeutic approaches.
- Observation without adjuvant therapy may be optimal for select stage I Wilms tumors to prevent treatment-related toxicity.