Oncocytoid Renal Cell Carcinoma After Neuroblastoma: A Report of Four Cases of a Distinct Clinicopathologic Entity

Department of Pathology, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
American Journal of Surgical Pathology (Impact Factor: 5.15). 08/1999; 23(7):772-80. DOI: 10.1097/00000478-199907000-00004
Source: PubMed


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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    • "Renal cell carcinoma after neuroblastoma are seen in long term survivors of childhood neuroblastoma [34]. Therefore it has been suggested that therapy for neuroblastoma plays a role in the pathogenesis of these renal carcinomas. "
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    • "In the previously cited study from Fleitz et al. [4], 10 of 16 RCC patients had received cyclophosphamide for treatment of NB. On the other hand, a specific relationship between neuroblastoma and subsequent RCC has been postulated, and NB survivors appear to be at risk for RCC even if they received no chemotherapy [9]. Post-NB RCC appears to have a distinctive pathologic appearance and has been recognized in the 2004 WHO renal tumor classification as a discrete entity [10]; however, there may be morphologic overlap with translocation RCCs [7]. "
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