Kwon Ji Eyon, Hwang Kihwan, Go Kyeong O, Wee Chan Woo, Kim In Ah, Kim Yu Jung, Choe Gheeyoung, Choi Byung Se, Han Jung Ho, Kim Chae Yong
Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam, Korea.
Seoul National University College of Medicine, Seoul, Korea.
Brain Tumor Res Treat. 2020 Oct;8(2):77-82. doi: 10.14791/btrt.2020.8.e18.
High-grade glioma (HGG) with primary leptomeningeal seeding (PLS) at initial diagnosis is rare. The purpose of this study was to identify its clinical features and to describe the clinical treatment outcomes.
We retrospectively reviewed the medical records of patients with HGG (World Health Organization grade III or IV) at our institution between 2004 and 2019, and patients with PLS at the initial diagnosis were enrolled in the study. Clinical features, such as the location of leptomeningeal seeding, surgical methods, and degree of resection, were sorted based on electronic medical records also containing performance scale, and hematological and serological evaluations. Radiological findings and immunohistochemical categories were confirmed. Furthermore, we sought to determine whether controlling intracranial pressure (ICP) via early cerebrospinal fluid (CSF) diversion increases overall survival (OS) after the initial diagnosis.
Of the 469 patients with HGG in our institution, less than 2% had PLS at the initial diagnosis. Most patients suffered from headache, diplopia, and dizziness. Pathological findings included 7 glioblastomas and 2 anaplastic astrocytomas. Seven of the 9 patients underwent CSF diversion. All patients were administered concurrent chemoradiotherapy (CCRT) with temozolomide, 89% of which started adjuvant temozolomide and 33% of which completed the six cycles of adjuvant temozolomide. The OS of patients with HGG and PLS was 8.7 months (range, 4-37), an extremely poor result compared to that of other studies. Also, the 1-year and 2-year OS rates were 44.4% and 16.7%, respectively.
Diagnosis and treatment of HGG with PLS are challenging. Aggressive control of ICP followed by early initiation of standard CCRT seems to be helpful in improving symptoms. However, despite aggressive treatment, the prognosis is poor. A multicenter trial and research may be necessary to create a standardized protocol for this disease.
初诊时伴有原发性软脑膜播散（PLS）的高级别胶质瘤（HGG）较为罕见。本研究旨在确定其临床特征并描述临床治疗结果。
我们回顾性分析了2004年至2019年间我院收治的HGG（世界卫生组织III级或IV级）患者的病历，纳入初诊时伴有PLS的患者。根据电子病历（其中还包含功能量表、血液学和血清学评估）对软脑膜播散的部位、手术方法和切除程度等临床特征进行分类。确认影像学检查结果和免疫组化类别。此外，我们试图确定通过早期脑脊液（CSF）分流控制颅内压（ICP）是否能提高初诊后的总生存期（OS）。
在我院469例HGG患者中，初诊时伴有PLS的患者不到2%。大多数患者有头痛、复视和头晕症状。病理检查结果包括7例胶质母细胞瘤和2例间变性星形细胞瘤。9例患者中有7例接受了脑脊液分流。所有患者均接受了替莫唑胺同步放化疗（CCRT），其中89%开始辅助使用替莫唑胺，33%完成了六个周期的辅助替莫唑胺治疗。HGG伴PLS患者的OS为8.7个月（范围4 - 37个月），与其他研究相比结果极差。此外，1年和2年OS率分别为44.4%和16.7%。
HGG伴PLS的诊断和治疗具有挑战性。积极控制ICP并尽早开始标准CCRT似乎有助于改善症状。然而，尽管进行了积极治疗，预后仍然很差。可能需要进行多中心试验和研究，以制定针对该疾病的标准化方案。
