Oncocytoid renal cell carcinoma after neuroblastoma: a report of four cases of a distinct clinicopathologic entity.
ABSTRACT Four children who developed oncocytoid renal cell carcinoma (RCC) after neuroblastoma are reported. One patient had multiple, bilateral RCCs. The mean age at time of diagnosis of RCC was 8.8 years (range, 5-13 years). The mean interval between neuroblastoma and RCC was 7.15 years (range, 3.1-11.5 years). The histologic findings of these RCCs did not fit within the spectrum of known renal epithelial neoplasms. Most of the neoplastic cells in all cases had eosinophilic, oncocytoid cytoplasm and were arranged in solid and papillary growth patterns. A subset of cells with reticular cytoplasm was also present. Immunohistochemical studies demonstrated keratins 8 and 18 in all neoplasms and keratin 20 in two cases. DNA ploidy analysis revealed that two of three neoplasms assessed were aneuploid. Cytogenetic studies revealed 45, XX, add or dup (7)(q32q36) in one neoplasm, and 83-89, XXXX, -1 ,-3, del (3)(q11.1q2?1), der(4)t(4;?22) (q32;q11.2), -14, -22 in a second tumor. Microsatellite polymerase chain reaction analysis detected no abnormalities in one neoplasm and allelic imbalance of chromosomes 2p31-32.2, 8p22, 9p22-24, 13q22, 20q13, and 22q11 in a second tumor. In case 4, two different RCCs excised 6 months apart were analyzed. The initial neoplasm showed allelic imbalance of chromosomes 2q31-32.2, 5q22, 5q31, 10p13-14, 13q22, 14q31, and 20q13. The subsequent neoplasm showed allelic imbalance of chromosomes 3p21.3, 14q31, and 20q13. The common presence of 14q31 and 20q13 abnormalities suggests that these two neoplasms were genetically related. In aggregate, these findings are distinctive, are not found in known types of RCC, and support the morphologic impression that oncocytoid RCC after neuroblastoma is a distinct clinicopathologic entity.
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Article: Pediatric renal cell carcinoma[Show abstract] [Hide abstract]
ABSTRACT: Renal cell carcinoma (RCC) comprises about 5% of pediatric renal neoplasms. It has been recognized as a second malignancy in multiple reports. It is generally symptomatic at diagnosis, and most children with RCC present with more locally advanced disease than do adults. Contemporary investigation of pediatric RCC has demonstrated that a large percentage of these tumors bear cytogenetic translocations involving the MiT family of transcription factors. Surgical therapy for these children resembles operative intervention for adult RCC, though debate continues about the precise role of lymph node dissection. There are no adequately powered studies to support conclusions about adjuvant or neoadjuvant chemotherapy for children with RCC. This may be ameliorated by a multi-institutional protocol which is enrolling patients.Journal of pediatric urology 06/2009; 5(4):308-14. DOI:10.1016/j.jpurol.2009.04.007 · 1.41 Impact Factor
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Article: [Pediatric renal cell carcinoma].[Show abstract] [Hide abstract]
ABSTRACT: Renal cell carcinoma in children and adolescents is rare and comprises only about 1% of renal tumors in this age group. Since the last WHO classification in 1997, new entities of renal tumors in young patients have been described and have been included into the new 2004 WHO renal cell carcinoma classification. Renal cell carcinoma associated with neuroblastoma comprises 2.5% of renal cell carcinoma in young patients. It occurs several years after neuroblastoma. A large proportion of these tumors show allelic imbalance of chromosomes 20q13, 2p31-32.2, 13q22 and 14q31. TFE3-translocation carcinomas correspond to approximately 20% of renal cell carcinomas in the pediatric and adolescent age group. Both translocations t(X;17)(p11.2;q25) and t(X;1)(p11.2;q21.2) result in immunohistochemically detectable TFE3 protein overexpression. By conventional morphology, TFE3-translocation carcinomas typically show prominent "voluminous" clear cytoplasm and partially papillary architecture. Even according to the revised 2004 WHO classification, in children and adolescents, far more renal cell carcinomas than in the adult age group are currently not classifiable but constitute a phenotypically heterogeneous group with ample potential for future renal cell carcinoma subtypes.Der Pathologe 08/2004; 25(4):324-7. · 0.64 Impact Factor