Clinical Characteristics of High-Grade Glioma with Primary Leptomeningeal Seeding at Initial Diagnosis in a Single Center Study

Abstract

Background 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. Methods 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. Results 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. Conclusion 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.

Leptomeningeal disease in glioblastoma: endgame or opportunity?

“…Mandel et al documented similar ndings, reporting that a combination of chemotherapy and radiotherapy led to prolonged overall survival of 7.7 months, which was increased compared to monotherapy or supportive care [7,24]. Kwon et al found that given the aggressive nature of LMD, patients who are diagnosed with LMD at the time of GBM diagnosis should undergo radiotherapy and chemotherapy earlier and more aggressively than in patients without LMD [25]. The low prevalence of LMD has proven to be an obstacle in examining the e cacy of various treatments.…”

“…Onuma et al noted that, while VP shunt surgery did not improve KPS in 10 GBM patients with hydrocephalus, 6 of 10 patients did experience subjective improvement in symptoms [38]. Kwon et al also noted that patients experienced less pain following CSF diversion [25]. Given that treatment at this stage of GBM is supportive and not curative, symptomatic improvement is signi cant for patients and should be considered by neuro-oncologists in concert with neurosurgeons.…”