Kapoor Ankita, Khushalani Nikhil, Gandhi Shipra
Internal Medicine, Rochester General Hospital, Rochester, USA.
Cutaneous Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, USA.
Cureus. 2020 Jun 15;12(6):e8637. doi: 10.7759/cureus.8637.
Renal cell carcinoma (RCC) can present with a myriad of clinical symptoms and signs. It is also notorious for its initial presentation with distant metastasis. We report a case of a 42-year-old male diagnosed with papillary RCC (PRCC) presenting with pleural and nodal metastases in the absence of a radiographically-detected tumor primary. PRCC was diagnosed on immunohistochemical analysis of the tissue from the pleura and mediastinal lymph nodes and confirmed by gene expression profiling studies. As per treatment guidelines for metastatic RCC, the patient was started on sunitinib with evidence of disease progression after two cycles and palliative care approach was recommended due to rapidly declining performance status. Prospective data on the optimal management of metastatic PRCC are lacking, but drugs used are similar to the treatment of clear cell carcinomas (vascular endothelial growth factor (VEGF) tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors) and checkpoint inhibitors. Further molecular study of these rare tumors is warranted to detect drivers of oncogenesis and identify targets for therapeutic intervention.
肾细胞癌（RCC）可表现出众多临床症状和体征。它还因其初发时即伴有远处转移而声名狼藉。我们报告一例42岁男性，诊断为乳头状肾细胞癌（PRCC），在未发现影像学检测到的肿瘤原发灶的情况下出现胸膜和淋巴结转移。PRCC通过对胸膜和纵隔淋巴结组织的免疫组化分析得以诊断，并经基因表达谱研究证实。根据转移性RCC的治疗指南，患者开始使用舒尼替尼治疗，两个周期后出现疾病进展迹象，鉴于患者体能状态迅速下降，建议采取姑息治疗方法。目前缺乏关于转移性PRCC最佳管理的前瞻性数据，但所使用的药物与透明细胞癌的治疗药物相似（血管内皮生长因子（VEGF）酪氨酸激酶抑制剂、雷帕霉素靶蛋白抑制剂）以及检查点抑制剂。对这些罕见肿瘤进行进一步的分子研究，对于检测肿瘤发生的驱动因素以及确定治疗干预靶点是必要的。
