Oncology 1 Unit, Istituto Oncologico Veneto IOV - IRCCS, Padua, Italy;
Oncology 1 Unit, Istituto Oncologico Veneto IOV - IRCCS, Padua, Italy.
Anticancer Res. 2023 May;43(5):2359-2365. doi: 10.21873/anticanres.16401.
BACKGROUND/AIM: Renal cell carcinoma (RCC) constitutes approximately 3% of all cancers. More than 60% of RCCs are detected incidentally; one-third of patients present with regional or distant metastases, and another 20-40% of patients develop metastases after radical nephrectomy. RCC can metastasize to any organ. In contrast, metastatic RCC (mRCC) without evidence of a primary tumor is extremely rare, with only a few reported cases.
We present a case of mRCC that initially presented with multiple liver and lymph node metastases but no primary renal lesion. An impressive response to treatment was achieved with a combination of immune checkpoint inhibitors and tyrosine kinase inhibitors. A clinical, radiological, and pathological diagnostic strategy, particularly in the context of a multidisciplinary team, are crucial for reaching a definitive diagnosis. This approach allows to select the appropriate treatment, making a huge difference for a mRCC due to its resistance to standard chemotherapy.
There are currently no guidelines available for mRCC without primary tumor. Nevertheless, a combination of TKI and immunotherapy could be the optimal first-line treatment if systemic therapy is required.
背景/目的:肾细胞癌(RCC)约占所有癌症的 3%。超过 60%的 RCC 是偶然发现的;三分之一的患者有局部或远处转移,另有 20-40%的患者在根治性肾切除术后发生转移。RCC 可以转移到任何器官。相比之下,没有原发性肿瘤证据的转移性 RCC(mRCC)极为罕见,仅有少数报道病例。
我们报告了一例 mRCC 病例,最初表现为多个肝和淋巴结转移,但无原发性肾病变。免疫检查点抑制剂和酪氨酸激酶抑制剂联合治疗取得了显著的疗效。对于多学科团队来说,临床、影像学和病理学的诊断策略,特别是在多学科团队的情况下,对于明确诊断至关重要。这种方法可以选择合适的治疗方法,对于 mRCC 来说意义重大,因为它对标准化疗有耐药性。
目前对于没有原发性肿瘤的 mRCC 尚无指南。然而,如果需要全身治疗,TKI 和免疫治疗的联合可能是最佳的一线治疗选择。