gms | German Medical Science

Objective: Spinal high-grade gliomas (sHGG) are a rare entity with poor prognosis. They are typically treated similar to cranial gliomas with surgical (partial) resection and radio-chemotherapy. However, the role of different molecular tumor profiles still remains unclear. We, therefore, aimed to assess the clinical courses of patients with sHGG at our institution in the recent decade with a focus on molecular tumor histology.

Methods: All consecutive patients with sHGG (WHO grad III or IV), treated at a single center between 2007 and 2017 were retrospectively reviewed. Only patients with available histopathological classification were included. Clinical, imaging and histopathological data were retrieved from the patients’ hospital records and tumor specific surgical as well as adjuvant treatments were noted. Functional outcome (McCormick Grade, KPI) was assessed at discharge and at follow-up visits. Overall survival (OS) was calculated.

Results: Six sHGG patients (five male, one female; age 49.3±19.7 years) were included in this analysis. All tumors were located in the thoracic spinal cord, most often lead to hypesthesia and paresis as the first symptoms and 66% showed contrast enhancement on MR-imaging. In all patients, surgical treatment consisted of a tumor biopsy or partial tumor resection. Histopathological classification revealed two WHO grade III sHGGs and four sHGGs with WHO grade IV. All tumors were negative for an IDH R132 mutation and 50% presented a H3.3 K27M- mutation. Of the three H3.3 K27M wildtype patients, two showed a methylation in the MGMT-promoter region. At discharge, a median McCormick grad of 3.2 (2-4) and a mean KPI of 58±18.3% were observed. All patients were treated with adjuvant radiotherapy 37.8±13.4 days after surgery and 50% of the patients received adjuvant chemotherapy (Temozolomid simultaneously to radiation, Bevazizumab (two patients) in the course). At 3-months follow-up, the median McCormick grade had decreased to 3.7 (3-4) and the KPI showed worsening to 43.3±8.16% as well. The mean OS of the sHGG patients was five months (1-13 months).

Conclusion: Treatment of sHGGs consists of partial surgical resection, followed by adjuvant radiotherapy and chemotherapy. The molecular genetic profiles of those rare tumors are still partially undetermined, but H3.3 K27M mutations seem to be relatively common. Prognosis is still very poor with a mean OS of less than six months and worsening of neurological symptoms during the course of disease.