| Renal cell carcinoma (RCC) associated with Xpl 1.2 translocation/TFE3 gene fusion (Xpl 1.2 RCC) was delineated as a distinct entity in the 2004 World Health Organization renal tumor classification. Its name is derived from this subtype of carcinoma which has gene fusion companied by the formation of chromosomes of Xp11.2 translocation. This subtype of RCC primarily affects children and adolescents more than adults, accounts for one third of pediatric RCCs,15% of patients under the age of 45 and about 1% of all renal tumors in adults. Xpl 1.2 RCC is insensitive to most of the radiotherapy and chemotherapy associated with a poorer prognosis and is reported more aggressive in adults than other subtypes of RCC. Now we choose radical nephrectomy as the primary treatment method for patients with Xp11.2 RCC.Renal cell carcinoma typically do not have capsule in histologic sense, but have pseudocapsule surrounding the tumor which is formed by compressed normal renal parenchyma and fiber texture. Research shows that complete pseudocapsules are usually found in cases of carcinoma which have smaller tumor volume, lower Fuhrman grades and grow slowly. Thus researchers consider that although TNM-staging do not involve psuedocapsule, cases with clear and complete psuedocapsule in preoperative imaging should be staged at early neoplasm staging. Through a series of pathologic studies of psuedocapsule, people realize that complete pseudocapsule means early neoplasm staging and less aggressivity, thus nephron sparing surgery, especially tumor enucleation could be used in these cases. Tumor enucleation is a kind of nephron sparing surgery, which resect the tumor along the natural space between the tumor psuedocapsule and normal renal parenchyma without removing macroscopic normal renal parenchyma. For these patients with small renal cell carcinomas which have limited tumor, complete psuedocapsule, non-infiltration and clear tumor boundaries, tumor enucleation is a reasonable surgical option. In previous studies of preoperative diagnosis and operation selection of renal cell carcinoma associated with Xp11.2 translocation, studies show that the CT value of Xpl 1.2 translocation renal carcinoma is significantly lower than that of renal cell carcinoma. To those children and young patients who have renal massive, if the CT value is lower than 50Hu, there is the possibility of Xpl 1.2 RCCs, which is a subtype of renal cell carcinoma. And since there is no effective method for estimating pathological subtype of Xpl 1.2 RCCs, now we treat Xp11.2 RCCs by using treatment principles of renal cell carcinoma.Methods:By studying the clinical and postoperative follow-up data of 32 patients with clear cell renal carcinoma and 27 patients with Xp11.2 RCC who were diagnosed by fluorescence in-situ hybridization polyclonal (FISH) assay from 2007-2015 retrospectively, we analysed pseudocapsule and other clinicopathological characteristics of the two subtypes of RCC and the survival of patients treated with radical nephrectomyas well as tumor enucleation.Results:Pseudocapsule incidence rate of Xp11.2 RCC (19/27,70.4%) was lower than that of ccRCC(clear cell renal carcinoma:ccRCC) (32/32,100%, P=0.001) while pseudocapsule integrity rate of Xp11.2 RCC (13/19,68.4%) was comparable with that of ccRCC (23/32,71.9%, P=1.000). The 5-year overall survival of patients with ccRCC treated with RN(radical nephrectomyas:RN) and TE(tumor enucleation:TE) was 86% and 81%, respectively (P=0.845). Eight patients with small Xp11.2 RCC had a good recorvery after NSS.Conclusions:Over half Xpl 1.2 RCC had pseudocapsules, whose integrity rate was comparable to that of ccRCC. A preliminary attempt to treat small Xpl 1.2 RCC with intact pseudocapsule by using NSS produced a favorable treatment outcome... |
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