研究垂体肿瘤初次诊断和垂体癌（PC）诊断时的临床病理特征，以及PC的治疗和预后，并确定与临床预后有关的潜在预后因素和治疗策略。在2021年5月搜索英语和法语报告诊断为有转移灶的PC的文章，不包括没有组织学证据并同时存在另一种癌症或不典型的垂体肿瘤的病例。共包括181篇文章，报告了207例病例，其中38％为皮质激素细胞瘤和29％为乳素细胞瘤。初次Ki67指数≥10％与初次诊断后较短的生存期有关（p=0.01）。早期出现转移的病例与较高的初始Ki67指数（p=0.01）和PC诊断后较短的生存期（p=0.001）有关。有趣的是，PC诊断后生存期较短的病例与初次诊断和PC诊断之间的时间较短有关（p=0.0006），并且其初始Ki67指数（p=0.003）和转移部位的Ki67指数（p=0.03）均较高。此外，PC诊断后生存期较长的病例更频繁地接受了系统治疗（p=0.0005）和局部治疗（p＜0.0001）。初次Ki67指数≥10％与不良预后有关，并且作为未来转移的一个有前途的早期标志物。其存在应导致加强监测和更及时的管理。临床医生不应犹豫使用局部治疗，无论是否使用系统治疗。

Investigate the clinicopathological characteristics at the initial diagnosis of the pituitary tumor and at pituitary carcinoma (PC) diagnosis, alongside with the management and outcomes of PCs, and identify potential prognostic factors and therapeutic strategies associated with the clinical outcome.Pubmed was searched in May 2021 for articles in English and French reporting PCs, of which diagnosis was made on the presence of metastases. The cases without histological proof and with either another cancer present or an atypical history for a pituitary tumor, were excluded. 181 articles reporting 207 cases were included, of which 38% corticotroph and 29% lactotroph carcinomas. An initial Ki67 index ≥10% was associated with shorter survival after the initial diagnosis (p=0.01). Cases with early metastases were associated with both higher initial Ki67 index (p=0.01) and shorter survival after PC diagnosis (p=0.001). Interestingly, cases with short survival after PC diagnosis were associated with shorter time between the initial diagnosis and PC diagnosis (p=0.0006), and had both higher initial Ki67 index (p=0.003), and higher Ki67 index of the metastasis (p=0.03). In addition, cases with long survival after PC diagnosis had received more frequently both systemic treatment after PC diagnosis (p=0.0005), and local treatment for metastases (p<0.0001). An initial Ki67 index ≥10% is associated with worse outcome, and appears as a promising early marker of future metastasis. Its presence should lead to an intensified surveillance and to a more timely management. Clinicians should not hesitate to use local treatment, independent of whether systemic treatment is used.