Departments of Pathology and Laboratory Medicine.
Urology, Indiana University School of Medicine, Indianapolis, IN.
Am J Surg Pathol. 2022 Sep 1;46(9):1171-1179. doi: 10.1097/PAS.0000000000001926. Epub 2022 Jun 13.
Sarcomatoid differentiation in chromophobe renal cell carcinoma (ChRCC) is a rare finding and a significant predictor of worse outcomes. When the sarcomatoid component overgrows the conventional component or is the only component on a biopsy, the differential diagnoses encompass a variety of entities. Therefore, we reviewed 22 sarcomatoid ChRCCs and characterized the immunophenotype. Given that renal carcinomas with sarcomatoid features may benefit from immune checkpoint inhibitor-based therapy we also assessed the programmed death-ligand 1 (PD-L1) (28-8) expression. DOG1, CD117, cytokeratin 7, and PAX8 were negative in 100%, 88%, 63%, and 44% of the sarcomatoid components, respectively. GATA3 was expressed in 31% of the conventional components and in 50% of the sarcomatoid components. One conventional and 3 sarcomatoid components expressed PD-L1. Sarcomatoid ChRCCs have a high propensity for metastases and cancer progression. Distant metastatic disease was seen in 73% of the cases and median survival in this cohort was <1 year. The sarcomatoid portion had increased expression of PD-L1 and frequent loss of expression of multiple immunohistochemical markers associated with ChRCC. Half of the sarcomatoid ChRCC exhibited GATA3 expression, 3 of which did not express PAX8.
嫌色细胞肾细胞癌（ChRCC）中的肉瘤样分化较为罕见，是预后不良的重要预测指标。当肉瘤样成分超过常规成分或在活检中是唯一成分时，鉴别诊断包括多种实体。因此，我们回顾了 22 例肉瘤样 ChRCC 并对其免疫表型进行了特征描述。鉴于具有肉瘤样特征的肾细胞癌可能受益于免疫检查点抑制剂治疗，我们还评估了程序性死亡配体 1（PD-L1）（28-8）的表达。DOG1、CD117、细胞角蛋白 7 和 PAX8 在 100%、88%、63%和 44%的肉瘤样成分中分别呈阴性。GATA3 在 31%的常规成分和 50%的肉瘤样成分中表达。1 例常规成分和 3 例肉瘤样成分表达 PD-L1。肉瘤样 ChRCC 具有较高的转移和癌症进展倾向。73%的病例出现远处转移疾病，该队列的中位生存时间<1 年。肉瘤样部分 PD-L1 表达增加，与 ChRCC 相关的多种免疫组化标志物表达频繁缺失。一半的肉瘤样 ChRCC 表现出 GATA3 表达，其中 3 例不表达 PAX8。
Zotero 插件