肺外神经内分泌肿瘤伴脑转移患者的管理与预后。

The Management and Outcomes of Patients with Extra-Pulmonary Neuroendocrine Neoplasms and Brain Metastases.

机构信息

Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester M20 4BX, UK.

Statistics Group, Digital Services, The Christie NHS Foundation Trust, Manchester M20 4BX, UK.

出版信息

Curr Oncol. 2022 Jul 20;29(7):5110-5125. doi: 10.3390/curroncol29070405.

DOI:10.3390/curroncol29070405

PMID:35877265

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9319979/

Abstract

BACKGROUND

Brain metastases (BMs) in patients with extra-pulmonary neuroendocrine neoplasms (EP-NENs) are rare, and limited clinical information is available. The aim of this study was to detail the clinicopathological features, management and outcomes in patients with EP-NENs who developed BMs.

METHODS

A retrospective single-centre analysis of consecutive patients with EP-NENs (August 2004-February 2020) was conducted. Median overall survival (OS)/survival from BMs diagnosis was estimated (Kaplan-Meier).

RESULTS

Of 730 patients, 17 (1.9%) had BMs, median age 61 years (range 15-77); 8 (53%) male, unknown primary NEN site: 40%. Patients with BMs had grade 3 (G3) EP-NENs 11 (73%), G2: 3 (20%), G1: 1 (7%). Eight (53%) had poorly differentiated NENs, 6 were well-differentiated and 1 was not recorded. Additionally, 2 (13%) patients had synchronous BMs at diagnosis, whilst 13 (87%) developed BMs metachronously. The relative risk of developing BMs was 7.48 in patients with G3 disease vs. G1 + G2 disease ( = 0.0001). Median time to the development of BMs after NEN diagnosis: 15.9 months (range 2.5-139.5). Five patients had a solitary BM, 12 had multiple BMs. Treatment of BMs were surgery ( = 3); radiotherapy ( = 5); 4: whole brain radiotherapy, 1: conformal radiotherapy (orbit). Nine (53%) had best supportive care. Median OS from NEN diagnosis was 23.6 months [95% CI 15.2-31.3]; median time to death from BMs diagnosis was 3.0 months [95% CI 0.0-8.3].

CONCLUSION

BMs in patients with EP-NENs are rare and of increased risk in G3 vs. G1 + G2 EP-NENs. Survival outcomes are poor, and a greater understanding is needed to improve therapeutic outcomes.

摘要

背景

肺外神经内分泌肿瘤（EP-NENs）患者的脑转移（BMs）较为罕见，目前临床相关信息有限。本研究旨在详细描述发生 BMs 的 EP-NENs 患者的临床病理特征、治疗方法和结局。

方法

对 2004 年 8 月至 2020 年 2 月期间连续就诊的 EP-NENs 患者进行回顾性单中心分析。采用 Kaplan-Meier 法估计 EP-NENs 患者的中位总生存期（OS）/BMs 诊断后的生存时间。

结果

730 例患者中，17 例（1.9%）发生 BMs，中位年龄 61 岁（范围 15-77 岁）；8 例（53%）为男性，原发 NEN 部位未知：40%。BMs 患者中 EP-NENs 分级 3 级（G3）11 例（73%），2 级（G2）3 例（20%），1 级（G1）1 例（7%）。8 例（53%）为低分化 NENs，6 例为中分化，1 例未记录。此外，2 例（13%）患者在诊断时同时发生 BMs，13 例（87%）患者发生异时性 BMs。G3 疾病患者发生 BMs 的相对风险是 G1+G2 疾病患者的 7.48 倍（ = 0.0001）。从 NEN 诊断到发生 BMs 的中位时间为 15.9 个月（范围 2.5-139.5）。5 例患者有单发 BMs，12 例患者有多发性 BMs。BMs 的治疗方法包括手术（ = 3）、放疗（ = 5）；其中 4 例为全脑放疗，1 例为适形放疗（眼眶）。9 例（53%）患者接受最佳支持治疗。从 NEN 诊断到死亡的中位 OS 为 23.6 个月[95%CI 15.2-31.3]；从 BMs 诊断到死亡的中位时间为 3.0 个月[95%CI 0.0-8.3]。