Raverot Gérald, Ilie Mirela Diana, Lasolle Hélène, Amodru Vincent, Trouillas Jacqueline, Castinetti Frédéric, Brue Thierry
Endocrinology Department, Reference Centre for Rare Pituitary Diseases HYPO, "Groupement Hospitalier Est" Hospices Civils de Lyon, Bron, France.
Lyon 1 University, Villeurbanne, France.
Nat Rev Endocrinol. 2021 Nov;17(11):671-684. doi: 10.1038/s41574-021-00550-w. Epub 2021 Sep 7.
Although usually benign, anterior pituitary tumours occasionally exhibit aggressive behaviour, with invasion of surrounding tissues, rapid growth, resistance to conventional treatments and multiple recurrences. In very rare cases, they metastasize and are termed pituitary carcinomas. The time between a 'classical' pituitary tumour and a pituitary carcinoma can be years, which means that monitoring should be performed regularly in patients with clinical (invasion and/or tumour growth) or pathological (Ki67 index, mitotic count and/or p53 detection) markers suggesting aggressiveness. However, although both invasion and proliferation have prognostic value, such parameters cannot predict outcome or malignancy without metastasis. Future research should focus on the biology of both tumour cells and their microenvironment, hopefully with improved therapeutic outcomes. Currently, the initial therapeutic approach for aggressive pituitary tumours is generally to repeat surgery or radiotherapy in expert centres. Standard medical treatments usually have no effect on tumour progression but they can be maintained on a long-term basis to, at least partly, control hypersecretion. In cases where standard treatments prove ineffective, temozolomide, the sole formally recommended treatment, is effective in only one-third of patients. Personalized use of emerging therapies, including peptide receptor radionuclide therapy, angiogenesis-targeted therapy and immunotherapy, will hopefully improve the outcomes of patients with this severe condition.
虽然垂体前叶肿瘤通常为良性，但偶尔也会表现出侵袭性行为，可侵犯周围组织、生长迅速、对传统治疗耐药并多次复发。在极少数情况下，它们会发生转移，被称为垂体癌。从“典型”垂体肿瘤发展为垂体癌可能需要数年时间，这意味着对于具有提示侵袭性的临床（侵袭和/或肿瘤生长）或病理（Ki67指数、有丝分裂计数和/或p53检测）标志物的患者，应定期进行监测。然而，尽管侵袭和增殖都具有预后价值，但在没有转移的情况下，这些参数无法预测预后或恶性程度。未来的研究应聚焦于肿瘤细胞及其微环境的生物学特性，有望改善治疗效果。目前，侵袭性垂体肿瘤的初始治疗方法通常是在专业中心重复手术或放疗。标准药物治疗通常对肿瘤进展无效，但可长期维持，以至少部分控制分泌过多。在标准治疗无效的情况下，唯一正式推荐的治疗药物替莫唑胺仅对三分之一的患者有效。包括肽受体放射性核素治疗、血管生成靶向治疗和免疫治疗在内的新兴疗法的个性化应用，有望改善这种重症患者的治疗效果。
