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Chromophobe Renal Cell Carcinoma: A Clinicopathologic Study of 203 Tumors in 200 Patients With Primary Resection at a Single Institution

Department of Pathology, Urology Service, Memorial Sloan-Kettering Cancer Center New York, NY 10065, USA.
The American journal of surgical pathology (Impact Factor: 4.59). 07/2011; 35(7):962-70. DOI: 10.1097/PAS.0b013e31821a455d
Source: PubMed

ABSTRACT Despite multiple studies, many clinicopathologic issues about chromophobe renal cell carcinoma (RCC) remain contentious; for example, its biological behavior-whether better or similar to papillary RCC, the incidence of sarcomatoid features, and whether pathologic features such as necrosis, nuclear grade, and tumor stage predict worse outcome. We studied 203 consecutive primary chromophobe RCCs resected at our institution in an attempt to answer these and other questions. The tumors showed significant progressive decrease in size and stage (P=0.047 and 0.001) from 1980 to 2000. Five patients had metastasis at presentation, and further disease-specific events (recurrence/metastasis/death due to disease) occurred in 8 more. Only 4 of 203 tumors had sarcomatoid features. Over median follow-up of 6.1 years (range, 0.1 to 18 y), 5-year and 10-year disease-specific events occurred in 3.7% (95% CI, 1.5%, 7.4%) and 6.4% (95% CI, 2.7%, 12.2%) patients. Outcomes showed significant association with tumor size, small-vessel invasion, sarcomatoid features, and microscopic necrosis (P≤0.05 each). pT stage or nodal metastasis tended to show some association, without reaching statistical significance (P=0.05 and 0.06, respectively). A modified tumor grading scheme, somewhat similar to that proposed recently, mitotic index, cytologic eosinophilia, and architecture, were not significantly associated with outcome. In conclusion, sarcomatoid differentiation is quite uncommon in chromophobe RCC. Tumor size, small-vessel invasion, sarcomatoid differentiation, and microscopic necrosis are the only features that are significantly associated with adverse outcome. On the basis of this long follow-up on a large number of cases, chromophobes seem to have better clinical outcomes than those reported for clear cell and papillary RCCs.

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    • "Chromophobe renal cell carcinoma (ChRCC) is a subtype of renal cell carcinoma (RCC), representing $5% of this heterogeneous group of cancers arising from the nephron (Stö rkel et al., 1997), with 3,000 new cases annually in the United States (Jemal et al., 2013). Although ChRCC typically exhibits an indolent pattern of local growth, with greater than 90% 10-year cancer-specific survival (Amin et al., 2002; Przybycin et al., 2011), aggressive features and metastasis can occur. ChRCC is associated with a distinct aneuploidy pattern (Speicher et al., 1994); however, genomewide evaluation of its somatic mutation spectrum has not been reported. "
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    • "By definition, chRCC is comprised of tumor cells with irregular nuclei with variation in nuclear size, and as a result, chRCCs would generally be assigned a Fuhrman grade of 3. Because of this issue, Paner et al. [17] recently proposed a three-tiered chromophobe tumor grade (CTG) system, that they report, demonstrates a positive association of CTG with both pathologic stage and outcome (Fig. 1). A subsequent study of 203 patients with chRCC utilized a modified grading scheme similar to that in the Paner et al. study; however, this scheme was not shown to be significantly associated with outcome [18]. Another study of 84 patients with chRCC utilized the CTG system of Paner et al. and found that CTG was not an independent predictor of outcome in multivariable analysis of non-sarcomatoid tumors [19]. "
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