Translocation renal cell carcinoma: lack of negative impact due to lymph node spread.

Department of Hematology Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio 45229-3039, USA.
Cancer (Impact Factor: 4.9). 05/2008; 112(7):1607-16.
Source: PubMed

ABSTRACT Pediatric renal cell carcinoma (RCC) is clinically distinct from adult RCC. Characterization of the unique biological and clinical features of pediatric RCC are required.
A retrospective review and biological analysis of all RCC cases presenting to Cincinnati Children's Hospital Medical Center (CCHMC) in the last 30 years was undertaken. Cases were classified according to the recent World Heath Organization morphologic classification and according to TFE3/TFEB status. A literature review of pediatric TFE+ cases was performed.
Eleven cases of RCC with clinical data were identified in our institutional review as follows: 6 clear cell, 2 papillary, 2 translocation, and 1 sarcomatoid. Upon reanalysis, 1 papillary and 1 sarcomatoid were confirmed, 1 case was "unclassified", and 8 of 11 (72.7%) had features consistent with translocation morphology. Of these 8, all demonstrated immunoreactivity for TFE3 (7 patients) or TFEB (1 patient) protein. In 3 cases, cytogenetics was available, each demonstrating confirmatory MiTF/TFE translocations. Seven of 8 TFE+ RCC patients presented with TNM Stage III/IV disease. Literature analysis confirmed a significant increase in advanced stage presentation in pediatric TFE+ RCC compared with TFE- RCC. Fourteen of fifteen (93.3%) patients with TFE+ stage III/IV RCC due to lymph node spread (N+ M(0)) remain disease free with a median and mean follow-up of 4.4 and 6.3 years, respectively (range, 0.3-15.5).
Translocation morphology RCC is the predominant form of pediatric RCC, associated with an advanced stage at presentation. Patients with TFE+ N+ M(0) RCC maintain a favorable short-term prognosis after surgery alone. Young RCC patients should be screened for translocation morphology, and the screening information should be considered when debating adjuvant therapy.

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    ABSTRACT: Context .- Eosinophilic renal neoplasms include a spectrum of solid and papillary tumors ranging from indolent benign oncocytoma to highly aggressive malignancies. Recognition of the correct nature of the tumor, especially in biopsy specimens, is paramount for patient management. Objective .- To review the diagnostic approach to eosinophilic renal neoplasms with light microscopy and ancillary techniques. Data Sources .- Review of the published literature and personal experience. Conclusions .- The following tumors are in the differential diagnosis of oncocytic renal cell neoplasm: oncocytoma, chromophobe renal cell carcinoma (RCC), hybrid tumor, tubulocystic carcinoma, papillary RCC, clear cell RCC with predominant eosinophilic cell morphology, follicular thyroid-like RCC, hereditary leiomyomatosis-associated RCC, acquired cystic disease-associated RCC, rhabdoid RCC, microphthalmia transcription factor translocation RCC, epithelioid angiomyolipoma, and unclassified RCC. In low-grade nonpapillary eosinophilic neoplasms, distinction between oncocytoma and low-grade RCC mostly rests on histomorphology; however, cytokeratin 7 immunostain may be helpful. In high-grade nonpapillary lesions, there is more of a role for ancillary techniques, including immunohistochemistry for cytokeratin 7, CA9, CD10, racemase, HMB45, and Melan-A. In papillary eosinophilic neoplasms, it is important to distinguish sporadic type 2 papillary RCC from microphthalmia transcription factor translocation and hereditary leiomyomatosis-associated RCC. Histologic and cytologic features along with immunohistochemistry and fluorescence in situ hybridization tests for TFE3 (Xp11.2) and TFEB [t(6;11)] are reliable confirmatory tests. Eosinophilic epithelial neoplasms with architecture, cytology, and/or immunoprofile not qualifying for either of the established types of RCC should be classified as unclassified eosinophilic RCC and arbitrarily assigned a grade (low or high).
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    12/2014; 24. DOI:10.1016/j.ijscr.2014.12.026
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