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儿童弥漫性中线胶质瘤脑室腹腔分流术后腹水产生和预后:病例系列研究。

Ascites production and prognosis after ventriculoperitoneal shunt for diffuse midline gliomas in children: A case series.

机构信息

Department of Clinical Laboratory, Guangdong Sanjiu Brain Hospital, Guangzhou, China.

State Key Laboratory of Brain and Cognitive Science, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China.

出版信息

Medicine (Baltimore). 2024 Oct 4;103(40):e39977. doi: 10.1097/MD.0000000000039977.

Abstract

RATIONALE

DMG is a highly invasive and lethal type of brain tumor. As these tumors progress, they often compromise the CSF circulation, leading to hydrocephalus. Ventriculoperitoneal shunt (VPS) is commonly employed to manage hydrocephalus; however, the complication of VPS-induced ascites, particularly in the presence of tumor cells, is a significant concern that merits attention.

PATIENT CONCERNS

This case series details 3 pediatric patients diagnosed with brainstem DMG harboring the H3 K27M mutation. Each developed hydrocephalus underwent VPS insertion. Post-operatively, all patients developed carcinomatous ascites with tumor cells detected within the ascitic fluid.

DIAGNOSES

All 3 patients were diagnosed with intra-abdominal metastasis of DMG H3K27M mutant cancer cells, each presenting with distinct complications.

INTERVENTIONS

Initially, the patients' primary head tumors responded to treatment, and their hydrocephalus resolved. However, some time after discharge, each patient developed malignant ascites and received palliative chemotherapy to control symptoms and improve quality of life.

OUTCOMES

Despite the interventions, all 3 patients died within 1 month of developing malignant ascites, with central respiratory failure being the direct cause of death.

LESSONS

These cases underscore the importance of continuous monitoring of both the CSF and ascitic fluid in patients with gliomas. Regular assessments of biochemical composition, cytology, and other diagnostic tests are crucial for early detection of disease progression. This proactive approach facilitates timely clinical judgment and intervention, potentially improving patient outcomes.

摘要

背景

弥漫性内生型桥脑胶质瘤(DMG)是一种高度侵袭性和致命性的脑肿瘤。随着肿瘤的进展,它们常常会影响脑脊液的循环,导致脑积水。脑室-腹腔分流术(VPS)常用于治疗脑积水;然而,VPS 引起的腹水并发症,特别是在存在肿瘤细胞的情况下,是一个值得关注的重要问题。

病例介绍

本病例系列详细介绍了 3 名被诊断为脑干 DMG 并携带 H3 K27M 突变的儿科患者。每个患者都因脑积水接受了 VPS 插入术。术后,所有患者均出现癌性腹水,腹水中检测到肿瘤细胞。

诊断

所有 3 名患者均被诊断为 DMG H3K27M 突变型癌细胞的腹腔内转移,每个患者都有不同的并发症。

干预措施

最初,患者的原发性脑部肿瘤对治疗有反应,脑积水得到缓解。然而,在出院后一段时间,每个患者都出现了恶性腹水,并接受姑息性化疗来控制症状和提高生活质量。

结果

尽管进行了干预,所有 3 名患者在出现恶性腹水后 1 个月内均死亡,中枢性呼吸衰竭是直接死亡原因。

教训

这些病例强调了对胶质瘤患者的脑脊液和腹水进行持续监测的重要性。定期评估生化成分、细胞学和其他诊断测试对于早期发现疾病进展至关重要。这种主动的方法有助于及时进行临床判断和干预,从而可能改善患者的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70f5/11460877/bdcd0e93e94b/medi-103-e39977-g001.jpg

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