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Review
. 2022 Sep 6:12:982089.
doi: 10.3389/fonc.2022.982089. eCollection 2022.

Surgical approaches to intramedullary spinal cord astrocytomas in the age of genomics

Andrew M Hersh  1 , George I Jallo  1   2 , Nir Shimony  1   3   4   5
Affiliations
Review

Surgical approaches to intramedullary spinal cord astrocytomas in the age of genomics

Andrew M Hersh et al. Front Oncol. .

Abstract

Intramedullary astrocytomas represent approximately 30%-40% of all intramedullary tumors and are the most common intramedullary tumor in children. Surgical resection is considered the mainstay of treatment in symptomatic patients with neurological deficits. Gross total resection (GTR) can be difficult to achieve as astrocytomas frequently present as diffuse lesions that infiltrate the cord. Therefore, GTR carries a substantial risk of new post-operative deficits. Consequently, subtotal resection and biopsy are often the only surgical options attempted. A midline or paramedian sulcal myelotomy is frequently used for surgical resection, although a dorsal root entry zone myelotomy can be used for lateral tumors. Intra-operative neuromonitoring using D-wave integrity, somatosensory, and motor evoked potentials is critical to facilitating a safe resection. Adjuvant radiation and chemotherapy, such as temozolomide, are often administered for high-grade recurrent or progressive lesions; however, consensus is lacking on their efficacy. Biopsied tumors can be analyzed for molecular markers that inform clinicians about the tumor's prognosis and response to conventional as well as targeted therapeutic treatments. Stratification of intramedullary tumors is increasingly based on molecular features and mutational status. The landscape of genetic and epigenetic mutations in intramedullary astrocytomas is not equivalent to their intracranial counterparts, with important difference in frequency and type of mutations. Therefore, dedicated attention is needed to cohorts of patients with intramedullary tumors. Targeted therapeutic agents can be designed and administered to patients based on their mutational status, which may be used in coordination with traditional surgical resection to improve overall survival and functional status.

Keywords: astrocytoma; biomarkers; genetic; intramedullary; resection; spinal cord; targeted therapy; tumor.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Sagittal T2-weighted MRI of pediatric patient with an intramedullary mass extending from C4-T3. Increased T2 signal is seen both cranially and caudally to the tumor. The lesion appears enhancing, although several areas of central non-enhancement consistent with necrosis are visible. Pathology was consistent with a Grade 2 astrocytoma.
Figure 2
Figure 2
Adult patient with an intramedullary astrocytoma seen on (A) sagittal T2-weighted MRI and (B) axial T2-weighed MRI extending from the C4 to T3 vertebral levels with effacement of the subarachnoid cerebrospinal fluid. The patient presented with lower limb paralysis and loss of bladder and bowel function. Intra-operatively, a subtotal resection was performed with removal of visible tumor; however, the tumor was noted to be infiltrative. Pathology revealed an H3K27M-altered diffuse midline glioma resulting in a WHO Grade 4 diagnosis, although the histological appearance resembled anaplastic astrocytoma.
Figure 3
Figure 3
Algorithm to guide surgical decision-making and adjuvant treatment in patients with intramedullary astrocytomas using genetic analysis of tumors. Created with BioRender.com.
See this image and copyright information in PMC

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