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      Renal Cell Carcinoma in Children: Experience of a Single Center

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          Objective: To evaluate the clinical features and outcome of children withrenal cell carcinoma (RCC). Patients and Methods: Eleven patients with RCC who were diagnosed between 1972 and 2004 were retrospectively analyzed. Clinical features, histopathology, treatment regimens and outcomes of the patients were evaluated. Results: The male/female ratio was 3:8, with a median age of 10 years. The stage distribution was as follows: 3 patients in stage I, 1 patient in stage II, 3 patients in stage IIIb, and 4 patients in stage IV. Five of 7 patients with stage II-IV received an actinomycin D-based regimen, one received a cisplatin-based regimen, and the other was given 5-fluorouracil (5-FU). In the last patient, interferon-α was given in combination with 5-FU; 1 of the patients on the actinomycin D regimen received interferon-α as well. All of the stage I patients are alive without disease. Three patients with stage IIIb, stage IV and stage II disease are alive without disease 8, 14 and 26 years after their diagnosis, respectively. The other stage IV and stage IIIb patients died of the disease. Conclusion: Nephroureterectomy is the main treatment modality, and it is sufficient for stage I patients. For patients with stage II-IV RCC, interferon-α and/or actinomycin D-based chemotherapy is the treatment of choice.

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          Local lymph node involvement does not predict poor outcome in pediatric renal cell carcinoma.

          Local lymph node involvement in adults with renal cell carcinoma (RCC) is associated with poor outcome. The prognostic significance of local lymph node involvement in children with RCC has not been studied systematically. A retrospective review of patients treated at St Jude Children's Research Hospital (Memphis, TN) and an extensive review of the medical literature were undertaken to evaluate the prognostic significance of local lymph node involvement in pediatric RCC. Thirteen patients with the diagnosis of RCC were treated at St. Jude since the hospital's inception in 1962. Four patients presented with lymph node-positive, distant metastasis-negative (N + M0) disease, and all 4 remain disease free after resection without adjuvant therapy (follow-up duration, 2-9 years). A systematic review of the literature including 243 pediatric patients with RCC revealed stage-specific survival rates of 92.5%, 84.6%, 72.7%, and 12.7% for Stage I-IV disease, respectively. Of 58 children with N + M0 RCC for whom outcome data were available, 42 (72.4%) were alive without disease at last follow-up. Among patients whose therapy could be discerned, those who received no adjuvant therapy fared as well (15 of 16 alive) as those who received various adjuvant treatments (22 of 31 alive). Children with lymph node-positive RCC in the absence of distant metastatic disease had a relatively favorable long-term prognosis, with survival rate nearly triple those of adult historical controls. Until highly effective therapies for RCC are identified, these children should not be exposed to adjuvant treatment. Further investigation of the biologic differences between adult and pediatric RCC is warranted. (c) 2004 American Cancer Society.
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            Renal cell carcinoma in children: a clinicopathologic study.

            To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC). The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts. Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients. RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease.
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              Renal-cell carcinoma in children: a different disorder from its adult counterpart?

              Renal-cell carcinoma (RCC) is a rare tumor in children. To address whether RCC in children differs from its adult counterpart, we report a series of 16 children with RCC (5 boys, 11 girls, mean age 9.6 years, range 3-19 years) presenting between 1979 and 1996 at three pediatric centers. Pathology showed papillary RCC in five patients (31%). Nonpapillary tumors were present in 11 (69%), of which nine were clear-cell type (56%), one was chromophobe-cell type (6%), and one was granular-cell type (6%). Cytogenetic studies were performed on four. In two tumors, normal karyotypes (45,XX or 45,XY) were found. In another, there were translocations: t(X;1), t(X;2) and t(6;14). In the fourth, analysis revealed 46,XX/46,X,t(X;17)(p11.2;q25),t(1;12). Several features in this series differ from those reported in adults. In adults, RCC is more frequent in males, is usually nonpapillary, and is characterized cytogenetically by deletions or rearrangements in the short arm of chromosome 3. In contrast, in our series there was no male predominance and a higher proportion of papillary tumors. In addition, two of four cytogenetically analyzed tumors had translocations involving the X chromosome. Translocations involving the Xp11.2 locus have been infrequently reported in both adults and children with papillary RCC. The higher frequency of papillary histology and the presence of translocations involving Xp.11.2 in two cases raise the possibility of a unique subtype of RCC in children.

                Author and article information

                Nephron Clin Pract
                Nephron Clinical Practice
                S. Karger AG
                January 2007
                29 November 2006
                : 105
                : 2
                : c58-c61
                aDepartment of Pediatric Oncology, Institute of Oncology, and Departments of bPediatric Surgery, and cPediatric Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
                97599 Nephron Clin Pract 2007;105:c58–c61
                © 2007 S. Karger AG, Basel

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                Page count
                Figures: 1, Tables: 1, References: 23, Pages: 1
                Self URI (application/pdf): https://www.karger.com/Article/Pdf/97599
                Original Paper

                Cardiovascular Medicine, Nephrology

                Renal cell carcinoma, Children, Outcome, Chemotherapy


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