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20 余年来三级中心肢端肥大症多模态管理的变化。

Changes in multi-modality management of acromegaly in a tertiary centre over 2 decades.

机构信息

Marseille Medical Genetics, Institut MarMaRa, INSERM, UMR1251, Aix Marseille Univ, Marseille, France.

Department of Endocrinology, Hôpital La Conception, Service d'Endocrinologie, Institut MarMaRa, APHM, Aix Marseille Univ, 147 Boulevard Baille, 13005, Marseille, France.

出版信息

Pituitary. 2024 Jun;27(3):294-302. doi: 10.1007/s11102-024-01387-y. Epub 2024 Mar 23.

Abstract

PURPOSE

Acromegaly is a rare disease associated with chronic multisystem complications. New therapeutic strategies have emerged in the last decades, combining pituitary transsphenoidal surgery (TSS), radiotherapy or radiosurgery (RXT) and medical treatments.

METHODS

This retrospective monocentric study focused on presentation, management and outcome of acromegaly patients diagnosed between 2000 and 2020, still followed up in 2020, with a minimum follow-up of 1 year, and comparison of the first vs. second decade of the study.

RESULTS

275 patients were included, 50 diagnosed before 2010 and 225 after 2010. 95% of them had normal IGF-1 levels (with or without treatment) at the last follow-up. Transsphenoidal surgery was more successful after 2010 (75% vs. 54%; p < 0.01), while tumor characteristics remained the same over time. The time from first treatment to biochemical control was shorter after 2010 than before (8 vs. 16 months; p = 0.03). Since 2010, RT was used less frequently (10% vs. 32%; p < 0.01) but more rapidly after surgery (26 vs. 53 months; p = 0.03). In patients requiring anti-secretory drugs after TSS, the time from first therapy to biochemical control was shorter after 2010 (16 vs. 29 months; p < 0.01). Tumor size, tumor invasiveness, baseline IGF-1 levels and Trouillas classification were identified as predictors of remission.

CONCLUSION

The vast majority of patients with acromegaly now have successful disease control with a multimodal approach. They reached biochemical control sooner in the most recent half of the study period. Future work should focus on those patients who are still uncontrolled and on the sequelae of the disease.

摘要

目的

肢端肥大症是一种罕见的疾病,与慢性多系统并发症有关。在过去的几十年中,出现了新的治疗策略,将垂体经蝶窦手术(TSS)、放疗或放射外科(RXT)和药物治疗相结合。

方法

这项回顾性单中心研究聚焦于 2000 年至 2020 年间诊断的肢端肥大症患者的表现、治疗和结局,这些患者在 2020 年仍在接受随访,随访时间至少为 1 年,并比较了研究的第一个和第二个十年。

结果

共纳入 275 例患者,其中 50 例患者在 2010 年前诊断,225 例患者在 2010 年后诊断。在最后一次随访时,95%的患者 IGF-1 水平正常(无论是否治疗)。2010 年后 TSS 的成功率更高(75% vs. 54%;p<0.01),而肿瘤特征在不同时间保持不变。2010 年后,从首次治疗到生化控制的时间更短(8 个月 vs. 16 个月;p=0.03)。自 2010 年以来,RT 的应用频率更低(10% vs. 32%;p<0.01),但术后应用更快(26 个月 vs. 53 个月;p=0.03)。对于 TSS 后需要抗分泌药物治疗的患者,2010 年后从首次治疗到生化控制的时间更短(16 个月 vs. 29 个月;p<0.01)。肿瘤大小、侵袭性、基线 IGF-1 水平和 Trouillas 分类被确定为缓解的预测因素。

结论

现在绝大多数肢端肥大症患者通过多模式治疗成功控制了疾病。他们在研究的最近一半时间达到了生化控制。未来的工作应该关注那些仍然未得到控制的患者和疾病的后遗症。

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