Aggressively recurring cervical intramedullary anaplastic astrocytoma in a pregnant patient

Background: Many patients with spinal juvenile pilocytic astrocytoma can experience prolonged remission after resection. However, some reports suggest that pregnancy may be associated with progression. Case Description: The authors provide an image report highlighting a case of rapid and aggressive transformation of an intramedullary astrocytoma of the cervical spine in a pregnant patient. Over the course of 1 year, the lesion progressed from a juvenile pilocytic astrocytoma to an anaplastic astrocytoma. Genetic testing revealed mutations associated with aggressive behavior. Conclusion: The case and associated imaging demonstrate the importance of close neurologic monitoring and counseling regarding risk of progression in pregnant patients with spinal gliomas.

Large Cervical Ependymoma: 2-Dimensional Operative Video

Spinal ependymomas compose approximately 60% of spinal gliomas, the predominance occurring within adults. These tumors are generally benign, and maximal surgical resection with neurological preservation is the surgical goal. This patient had a large upper cervical ependymoma, which was approached through a cervical laminotomy. The surgical resection of this lesion demonstrates the principles of pial venous plexus preservation during posterior midline raphe identification and dissection. Gross total surgical resection was achieved with the preservation of the patient's baseline neurological function. The patient gave informed consent for surgery and video recording. Institutional review board approval was deemed unnecessary. Used with permission from Barrow Neurological Institute, Phoenix, Arizona.

MPC-16 RAPID PROGRESSIVE SPINAL DIFFUSE MIDLINE GLIOMA, A CASE REPORT

A 17-year-old boy presented with a 2-week history of lower back pain, progressive gait difficulty and sensory deficit of bilateral lower limbs. Magnetic resonance imaging of neuroaxis showed intramedullary tumor with spinal cord expansion from Th12 to L2 and irregular areas of enhancement. Emergent laminoplasty and biopsy was performed. Histopathological examination showed small atypical cells, but most cells had too much degeneration and necrosis to confirm the diagnosis definitively. Leptomeningeal dissemination caused conscious disturbance, nuchal rigidity and epilepsy. 2 weeks after decompression, we performed cordotomy again for advanced diagnosis, to be found diffuse midline glioma, H3K27M mutant by immunohistopathological examination and DAN sequence. He was treated with combination of whole brain and spine radiation therapy and chemotherapy with temozolomide and bevacizumab. He is still alive over 6 months. The clinical significance of H3K27M mutant in spinal gliomas is unclear. Further examinations are needed.

OS2.2 Chemotherapy for spinal gliomas in adults

BACKGROUND Chemotherapy is a treatment option in patients diagnosed with anaplastic gliomas or glioblastomas of the spinal cord, or with recurrent lower graded WHO spinal gliomas that are no longer amenable to local treatment. The low incidence of spinal cord gliomas, particularly in adults, limits the ability to perform clinical trials. The role of chemotherapy in these tumors has remained unclear. MATERIAL AND METHODS We performed a retrospective study of 22 patients diagnosed with spinal gliomas who were treated with chemotherapy at any time during the disease course. Benefit from chemotherapy was estimated by applying Response assessment in neuro-oncology criteria. Data on radiotherapy, as well as the number of neurosurgical interventions were taken into consideration. RESULTS Most patients were diagnosed with astrocytoma WHO grade I-IV (N=14), the remaining patients were diagnosed with ependymoma (N=8). Median follow-up from start of chemotherapy was 92 months (95% CI, 72.6–111.4). The O6-methylguanyl-DNA-methyltransferase(MGMT)promoter methylation status was available in tumors of 12 patients: 9 tumors (75%) had an unmethylated MGMTpromoter. More than 50% of the patients had more than one neurosurgical intervention. After prior surgery 10 patients in the first-line setting had chemotherapy combined with radiotherapy, while 3 patients received chemotherapy only. The remaining 9 patients had initially received radiation therapy and chemotherapy was given at time of recurrence. In patients diagnosed with astrocytoma mainly temozolomide (TMZ) was applied (N=10), while one patient received CCNU and three patients had combination chemotherapy. Patients diagnosed with ependymoma had hydroxyurea (N=1), CCNU (N=1), TMZ (N=3) or combination chemotherapy (N=3). In the group of patients who had chemotherapy combined with radiation, response rates were as follows: anaplastic astrocytoma 3 stable diseases (SD), glioblastoma 1 complete response (CR) and 1 SD, and anaplastic ependymoma 1 SD. After chemotherapy in the group of patients previously irradiated, the following response rates were observed: 1 SD in pilocytic astrocytoma, 1 SD in diffuse astrocytoma, 3 SD in myxopapillary ependymoma, and 2 SD and 1 partial response (PR) in anaplastic ependymoma. All other patients experienced progressive disease. There was no indication for a favorable prognostic role ofMGMTpromoter methylation. CONCLUSION Spinal cord gliomas represent a heterogeneous group of tumors. Survival outcomes in response to chemotherapy in adult spinal glioma patients vary substantially, but individual patients appear to derive benefit from chemotherapy.