Temozolomide for malignant primary spinal cord glioma: an experience of six cases and a literature review

Abstract

Malignant primary spinal cord gliomas (PSCGs) are rare, and the optimal treatment for these lesions remains controversial. We report herein treatment outcomes of six malignant PSCGs managed with temozolomide (TMZ)-based multidisciplinary treatment. TMZ was administered concomitantly with fractionated radiotherapy for two newly diagnosed primary spinal cord glioblastoma multiforme (GBM), followed by adjuvant chemotherapy with TMZ. For one anaplastic astrocytoma (AA) and one anaplastic ependymoma (AEPN), TMZ was given as adjuvant therapy at first recurrence. One malignantly transformed ependymoma (EPN) and one malignantly transformed diffuse astrocytoma (DA) were treated with TMZ after radiotherapy at second recurrence. Two patients with newly diagnosed GBM died, 12 and 16 months, respectively, after being treated with TMZ, during and after radiation therapy. One patient with AA and one with malignantly transformed EPN showed good response to salvage therapy with TMZ and had stable disease 21 and 20 months, respectively, after TMZ treatment. One patient with recurrent AEPN and one with malignantly transformed DA died from uncontrolled progression of the lesions despite TMZ chemotherapy. Three patients developed grade 1 or 2 neutropenia, anemia, and infection. Nonhematologic toxicities occurred in all patients; however, they were below grade 3 in severity. TMZ treatment may have a positive effect on control of malignant PSCGs and survival for some patients. Specifically, treatment with TMZ during and after radiation therapy might provide survival benefit to patients with primary spinal cord GBM. A multicenter cooperative investigation for a large-scale study on malignant PSCGs may be required.

A retrospective observational study on the treatment outcomes of 26 patients with spinal cord astrocytoma including two cases of malignant transformation

“…A prior report found survival benefits of temozolomide after radiotherapy in six patients with malignant primary spinal cord tumors [10]. Fakhreddine et al also reported that among patients with infiltrative astrocytomas, chemotherapy was significantly associated with improved PFS yet not OS on multivariate analysis [17].…”

“…Intramedullary spinal cord astrocytomas account for 6-8 % of all primary spinal cord tumors [5][6][7], indicating their rarity. Few investigations into the pathologic features, postoperative outcomes, surgical experience, or chemotherapy regimens have been published for these tumors; [1,[8][9][10] therefore, information is lacking on the common clinico-radio-pathologic features of spinal cord astrocytomas. Low-grade histologic findings, which include pilocytic astrocytomas, diffuse astrocytomas, and pilomyxoid astrocytomas, are more common than highgrade findings, such as anaplastic astrocytomas (AA) and glioblastoma (GB) [4,11,12].…”

“…Additional radiation therapy is recommended for cases of partial resection. There is currently no gold standard treatment for malignant primary spinal cord glioma [10].…”

Anaplastic Ganglioglioma: A Very Rare Intramedullary Spinal Cord Tumor

“…There is only one report of a long-term survivor in the pediatric population (table 1, gray shading), and this patient received combined adjuvant radiochemotherapy treatment [4]. Therefore, combined treatment with percutaneous irradiation and adjuvant concomitant chemotherapy with temozolomide was planned based on this case report and some prior evidence concerning treatment of low- and high-grade spinal cord astrocytomas [4,16,17]. …”

Radiotherapy of spinal cord gliomas

“…There is an extremely limited number of retrospective series and single case reports, suggesting that regimens used in the treatment of cerebral gliomas may be beneficial in spinal cord gliomas [34][35][36][37][38]. To the best of our knowledge, this is the largest study reporting the results of simultaneous chemoradiation in spinal cord gliomas.…”