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| |  AAP: Is Chemotherapy Helpful for Children with Cystic Partially Differentiated Nephroblastoma? By Maria Bishop Special to DG News BOSTON, MA -- October 21, 2002 -- Children with stage-II cystic partially differentiated nephroblastoma (CPDN) -- a rare variant of Wilms' tumor -- can see significant gains with adjuvant therapy, noted researchers here October 20 at the annual meeting of the American Academy of Pediatrics (AAP). CPDN is believed to have a more favourable outcome than standard nephroblastoma, but the need for chemotherapy has remained unproven. In this study, researchers from across the United States examined the outcome of children with CPDN who underwent nephrectomy and were treated with either vincristine- and dactinomycin-based chemotherapy (with or without doxorubicin) or no chemotherapy. According to lead author Martin L. Blakely, MD, from the department of paediatric surgery at University of Texas Health Science Center, in Houston, Texas, all patients were registered on the National Wilms Tumor Study (NWTS), and data were collected prospectively. All patients had central review by the NWTS Group (NWTSG) Pathology Center to confirm the diagnosis of CPDN. Demographic characteristics of the patients, and tumour characteristics were recorded, including surgical and medical therapy, local or distant recurrence and survival. Outcome is generally favourable for patients presenting early with CPDN, with long-term disease-free survival expected. Out of the 5,100 children enrolled in NWTSG studies III-V, (January 1981 to July 2000), 26 patients had a diagnosis of CPDN. Thirteen patients received chemotherapy and 13 patients did not in a non-randomised fashion. Five patients in the "no chemotherapy" group did not consent to being and the study, and are not included. The age at diagnosis was similar for those receiving chemotherapy compared to those not receiving chemotherapy (1.5 versus 1.0 years), as was tumour size (9.4 versus 9.9 cm). In the chemotherapy group, the stage distribution was as follows: stage I (n=10), stage II (2), and stage V (1). In the "no chemotherapy" group, all patients were stage I. All patients had complete tumour resection. Follow-up data were available for all patients; the median length of follow up was 6.2 years. No patient had disease progression or recurrence. For children with stage-I tumours, the outcome was similar for those treated with nephrectomy alone or nephrectomy plus chemotherapy. Children with stage-II disease, noted Dr. Blakely, did well with adjuvant therapy, without evidence of recurrence.
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