Heterogeneity of Disease Classified as Stage III in Wilms Tumor: A Report From the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)
- Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy)
- Department of Radiology/Radiotherapy, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy)
- Pediatric Oncology, Ospedale dei Bambini G. di Cristina, Palermo (Italy)
- Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy)
- Pediatric Department, Ospedale Infantile Regina Margherita, Torino (Italy)
- Pediatric Unit, Ospedali Riuniti Bergamo, Bergamo (Italy)
- Pediatric Oncology, Pediatric Department, II University, Napoli (Italy)
- Pediatric Hematology and Oncology Unit 'Lalla Seragnoli', Bologna University, Bologna (Italy)
- Department of Hematology and Oncology, Istituto G. Gaslini, Genova (Italy)
- Pediatric Hematology and Oncology, Catania University, Catania (Italy)
- Pediatric Surgery Unit, Urology, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy)
Purpose: We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems. Methods and Materials: Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT). Results: Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% {+-} 4% and 92% {+-} 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% {+-} 7%, as opposed to 98% {+-} 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% {+-} 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate. Conclusions: This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed.
- OSTI ID:
- 22055962
- Journal Information:
- International Journal of Radiation Oncology, Biology and Physics, Vol. 82, Issue 1; Other Information: Copyright (c) 2012 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved.; Country of input: International Atomic Energy Agency (IAEA); ISSN 0360-3016
- Country of Publication:
- United States
- Language:
- English
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