Department of Cancer Medicine, Institut Gustave Roussy, University of Paris Sud, 39 rue Camille Desmoulins, F-94800 Villejuif, France. christophe.massard@ igr.fr
Nat Rev Clin Oncol. 2011 Nov 1;8(12):701-10. doi: 10.1038/nrclinonc.2011.158.
Carcinomas of an unknown primary origin (CUP) account for 3-5% of all malignancies and are thus among the ten most-frequent cancers worldwide. Having a specific and unique phenotype of early and usually aggressive metastatic dissemination with no identifiable primary tumor, CUP are a challenge for physicians. The diagnostic workup of patients with CUP includes a careful clinical and extensive histopathological examination, as well as the use of imaging techniques. CUP can be divided into favorable and unfavorable subsets. Patients with unfavorable CUP subsets have a poor prognosis with a median survival of approximately 8 months; the optimal chemotherapy regimen for these patients remains to be determined. Although studies have focused on the introduction of new cytotoxic agents with broad-spectrum clinical activity (such as gemcitabine, irinotecan, and taxanes), no randomized trial has provided clear evidence of a survival benefit. Molecular targeted therapies that are approved for other solid tumors are now considered for the treatment of patients with CUP. Molecular diagnostic tools, such as DNA microarray analysis, could help in the search for 'lost' CUP origins. In this Review, we describe the clinical evaluation of patients with CUP, and discuss treatment strategies and outcomes of patients with various CUP subsets.
不明原发灶癌（CUP）占所有恶性肿瘤的 3-5%，因此是全球最常见的十种癌症之一。由于缺乏可识别的原发性肿瘤，CUP 具有早期和通常具有侵袭性转移扩散的特定且独特的表型，这对医生来说是一个挑战。CUP 患者的诊断包括仔细的临床和广泛的组织病理学检查，以及使用成像技术。CUP 可以分为有利和不利亚组。具有不利 CUP 亚组的患者预后较差，中位生存期约为 8 个月；这些患者的最佳化疗方案仍有待确定。尽管研究集中在引入具有广谱临床活性的新型细胞毒性药物（如吉西他滨、伊立替康和紫杉烷类药物）上，但没有随机试验提供生存获益的明确证据。现在，已批准用于其他实体瘤的分子靶向疗法被认为可用于治疗 CUP 患者。DNA 微阵列分析等分子诊断工具可帮助寻找“丢失”的 CUP 起源。在这篇综述中，我们描述了 CUP 患者的临床评估，并讨论了各种 CUP 亚组患者的治疗策略和结果。
