| Abstract: |
Purpose: Children who survive cancer are at more than 19-fold increased risk of developing another malignancy. Renal cell carcinoma (RCC) occurring as a secondary malignancy is uncommon. Translocation RCC, bearing TFE3 or TFEB gene fusions, are recently recognized entities for which risk factors have not been identified. Patients and Methods: We describe the clinical, pathologic, cytogenetic, and molecular data on six translocation RCCs that arose in five young patients who had received chemotherapy. Results: The ages at time of diagnosis of the RCC ranged from 6 to 22 years. Histologically, these tumors showed typical features previously described for translocation RCCs. At the molecular level, three tumors contained the ASPL-TFE3 fusion, two contained Alpha-TFEB, and one contained PRCC-TFE3. The intervals between chemotherapy and the diagnosis of RCC ranged from 4 to 13 years. The indications for the antecedent chemotherapy were varied and included acute promyelocytic leukemia, acute myeloid leukemia with t(9;11), bilateral Wilms' tumor, systemic lupus erythematosus, and conditioning regimen of bone marrow transplant for Hurler's syndrome. Only the latter patient had also received radiation. Hence, among 39 genetically confirmed translocation RCCs in our personal experience, six (15%) have arisen in patients who had received cytotoxic chemotherapy. Conclusion: Cytotoxic chemotherapy may predispose to the development of renal translocation carcinomas. © 2006 by American Society of Clinical Oncology. |
| Keywords: |
adolescent; adult; cancer chemotherapy; child; clinical article; controlled study; acute granulocytic leukemia; gene translocation; genetics; prednisone; cisplatin; doxorubicin; antineoplastic agents; cancer radiotherapy; cytarabine; antineoplastic agent; treatment indication; carboplatin; etoposide; neoplasm proteins; cytogenetics; cyclophosphamide; dexamethasone; vincristine; histology; kidney carcinoma; kidney neoplasms; oncogene; kidney tumor; carcinoma, renal cell; tumor protein; daunorubicin; promyelocytic leukemia; gene fusion; translocation, genetic; systemic lupus erythematosus; neoplasms, second primary; second cancer; bone marrow transplantation; tioguanine; teniposide; thymocyte antibody; nephroblastoma; tfe3 gene; hurler syndrome; basic helix loop helix leucine zipper transcription factor; tfe3 protein, human; basic helix-loop-helix leucine zipper transcription factors; tfeb protein, human; alveolar soft part sarcoma gene; pediatric renal cell carcinoma gene
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