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胰腺导管腺癌合并脑膜转移:长寿的表现。

Leptomeningeal disease in pancreas ductal adenocarcinoma: A manifestation of longevity.

机构信息

Department of Medicine, Memorial Sloan Kettering Cancer Center, USA.

Department of Neurology, Memorial Sloan Kettering Cancer Center, USA.

出版信息

Pancreatology. 2021 Apr;21(3):599-605. doi: 10.1016/j.pan.2021.02.003. Epub 2021 Feb 6.

Abstract

BACKGROUND

/Objectives: Pancreatic adenocarcinoma (PDAC) metastatic to the leptomeninges is a rare and lethal event. Leptomeningeal disease (LMD) research is limited in PDAC, and insights into clinical descriptors, possible disease predictors, and treatment strategies is necessitated.

METHODS

Memorial Sloan Kettering databases were queried with Institutional Review Board approval to identify patients with LMD and PDAC treated between January 2000 and June 2020. Medical record review was used to abstract clinical, genomic, pathologic, and radiographic data. Overall survival was calculated from date of PDAC diagnosis to date of death. Previously published literature on LMD from PDAC was reviewed.

RESULTS

Four patients with LMD from PDAC were identified, two males and two females. Age at diagnosis ranged from 57 to 68 years. All four patients had predominant lung metastasis and a relatively low burden of intra-abdominal disease. Somatic testing indicated alterations typical of PDAC and no PDAC defining pathogenic germline mutations were identified. An extended clinical course prior to LMD diagnosis was observed in all patients, ranging from 16 to 148 months. Upon diagnosis of LMD, three patients elected for supportive care and one patient received a limited course of craniospinal radiation. The median survival following diagnosis of LMD was 1.6 months (range 0.5-2.8 months).

CONCLUSIONS

LMD from PDAC is a rare occurrence that may be more frequent in patients with lung metastasis and/or a more indolent clinical course. Following diagnosis of LMD, prognosis is poor, and survival is short. New treatment strategies for this manifestation of PDAC are needed.

摘要

背景

/目的:胰腺癌(PDAC)向软脑膜转移是一种罕见且致命的事件。PDAC 中脑膜疾病(LMD)的研究有限,需要深入了解临床特征、可能的疾病预测因素和治疗策略。

方法

经机构审查委员会批准,对 2000 年 1 月至 2020 年 6 月期间接受 LMD 和 PDAC 治疗的患者,使用 Memorial Sloan Kettering 数据库进行查询。使用病历回顾法提取临床、基因组、病理和影像学数据。从 PDAC 诊断日期到死亡日期计算总生存期。回顾了 PDAC 引起的 LMD 的先前发表的文献。

结果

确定了 4 例 PDAC 引起的 LMD 患者,2 例男性,2 例女性。诊断时的年龄范围为 57 至 68 岁。所有 4 例患者均有主要的肺部转移,且腹腔内疾病负担相对较低。体细胞检测显示出 PDAC 的典型改变,未发现 PDAC 定义的致病性种系突变。所有患者在 LMD 诊断前都有较长的临床病程,范围为 16 至 148 个月。在 LMD 诊断时,3 例患者选择了支持性治疗,1 例患者接受了有限疗程的颅脊髓放疗。LMD 诊断后的中位生存期为 1.6 个月(范围 0.5-2.8 个月)。

结论

PDAC 引起的 LMD 是一种罕见的疾病,可能在有肺部转移和/或更惰性的临床病程的患者中更为常见。在 LMD 诊断后,预后较差,生存期较短。需要新的治疗策略来治疗 PDAC 的这种表现。

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