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Clear cell papillary renal cell carcinoma (CCPRCC) is a recently recognized subtype of renal cell carcinoma entity, however, little is known about its clinical features. In the present study, 26 cases of CCPRCC were screened out from two institutions. The patient data, tissue pathology, immunohistochemical phenotype, computed tomographic images and survival analysis were studied. The mean age was 53.3 years and the average tumor size was 2.5 cm. A total of 17 patients' body mass indexes were higher than the normal level. A total of 11 patients had hypertension and 6 patients had a smoking history. Histopathologically, all cases of CCPRCC exhibited a tubular and papillary architecture, small to medium-sized cuboidal tumor cells with clear cytoplasms, and a low Fuhrman nuclear grade. All tumors were encapsulated by variably thick fibrous capsules. Immunohistochemistry showed diffuse and moderate to strong cytoplasmic staining for CK7, CA IX and vimentin, but negative for AMACR and CD10 (sometimes focally positive) in all cases. According to the results of Ki67 labeling index, the expression of Ki67 in CCPRCC was much lower than that in clear cell renal cell carcinoma (CCRCC) (2.19 vs. 7.07%, P&lt;0.001) and that in papillary renal cell carcinoma (PRCC) (2.19 vs. 6.65%, P&lt;0.001). Radiographically, the tumors were shown as small masses with smooth contour and mixed enhancement pattern. The multiphasic attenuation curve for CCPRCC, like that for CCRCC, increased in the corticomedullary phase markedly and decreased in the nephrographic phase and excretory phase gradually. At a median follow-up period of 50 months, no cancer-specific death or tumor recurrence was observed. Considering the favorable prognosis of CCPRCC, preoperative biopsy in order to make clear the diagnosis is particularly important. In light of the present findings, partial nephrectomy for patients with CCPRCC is recommended. If the patients cannot tolerate surgery, closed monitoring or radiofrequency ablation may be considered.

Clinical features and survival analysis of clear cell papillary renal cell carcinoma: A 10‑year retrospective study from two institutions