Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy.
OBJECTIVE
Cystic partially differentiated nephroblastoma (CPDN) is a rare variant of Wilms' tumor thought to be more favorable than standard nephroblastoma. The purpose of this report is to examine the outcome of children with CPDN, after nephrectomy, treated with vincristine and dactinomycin based chemotherapy (+/- doxorubicin) or no chemotherapy.


METHODS
Patients were registered with the National Wilms' Tumor Study Group (NWTSG) and data were collected prospectively. All patients had central review by the NWTSG Pathology Center to confirm the diagnosis of CPDN.


RESULTS
Twenty-one patients were identified with a diagnosis of CPDN. Thirteen patients received chemotherapy, and 8 patients did not. In the chemotherapy group the stage distribution was as follows: stage I (n = 10), stage II (n = 2), stage V (n = 1). In the no-chemotherapy group, all 8 patients were stage I. All patients had complete tumor resection. There were no cases of disease progression or recurrence in any patient. In patients receiving chemotherapy, 30% (n = 4) had toxicities causing dose reduction.


CONCLUSIONS
The outcome of patients with CPDN is favorable with 100% survival rate and no recurrences. For stage I patients, treatment with complete tumor resection appears to be as efficacious as nephrectomy plus chemotherapy. Stage II patients also have excellent outcome when treated with tumor resection and postoperative vincristine and dactinomycin.
Blakely
Martin L
ML
Department of Surgery, University of Texas, Houston, TX, USA.
Shamberger
Robert C
RC
Norkool
Patricia
P
Beckwith
J Bruce
JB
Green
Daniel M
DM
Ritchey
Michael L
ML
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002454:Cell Differentiation / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D003609:Dactinomycin / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000175:diagnosis / Q000188:drug therapy* / Q000601:surgery; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D009396:Wilms Tumor / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology* / Q000601:surgery
D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin