Chordoma is a low-grade notochordal tumor of the skull base, mobile spine and sacrum which behaves malignantly and confers a poor prognosis despite indolent growth patterns. These tumors often present late in the disease course, tend to encapsulate adjacent neurovascular anatomy, seed resection cavities, recur locally and respond poorly to radiotherapy and conventional chemotherapy, all of which make chordomas challenging to treat. Extent of surgical resection and adequacy of surgical margins are the most important prognostic factors and thus patients with chordoma should be cared for by a highly experienced, multi-disciplinary surgical team in a quaternary center. Ongoing research into the molecular pathophysiology of chordoma has led to the discovery of several pathways that may serve as potential targets for molecular therapy, including a multitude of receptor tyrosine kinases (e.g., platelet-derived growth factor receptor [PDGFR], epidermal growth factor receptor [EGFR]), downstream cascades (e.g., phosphoinositide 3-kinase [PI3K]/protein kinase B [Akt]/mechanistic target of rapamycin [mTOR]), brachyury—a transcription factor expressed ubiquitously in chordoma but not in other tissues—and the fibroblast growth factor [FGF]/mitogen-activated protein kinase kinase [MEK]/extracellular signal-regulated kinase [ERK] pathway. In this review article, the pathophysiology, diagnosis and modern treatment paradigms of chordoma will be discussed with an emphasis on the ongoing research and advances in the field that may lead to improved outcomes for patients with this challenging disease.

Post-operative CSF leaks are a known complication of spine surgery in general, and patients undergoing surgical intervention for spinal tumors may be particularly predisposed due to the presence of intradural tumor and a number of other factors. Post-operative CSF leaks increase morbidity, lengthen hospital stays, prolong immobilization and subject patients to a number of associated complications. Intraoperative identification of unintended durotomies and effective primary repair of dural defects is an important first step in the prevention of post-operative CSF leaks, but in patients who develop post-operative pseudomeningoceles, durocutaneous fistulae or other CSF-leak-related sequelae, early recognition and secondary intervention are paramount to preventing further CSF-leak-related complications and achieving the best patient outcomes possible. In this article, the incidence, risk factors and complications of CSF leaks after spine tumor surgery are reviewed, with an emphasis on avoidance of post-operative CSF leaks, early post-operative identification and effective secondary intervention.

BACKGROUND: Neural components of the fibrous filum terminale (FT) are well known but are considered as embryonic remnants without functionality. OBJECTIVE: To investigate the ultrastructure of human FT specimens for sensory nerve endings and record paraspinal muscle activity on electrostimulation of the FT. METHODS: We prospectively investigated a cohort of 53 patients who underwent excision of the FT for the treatment of tethered cord syndrome. Surgical FT specimens were investigated by light and transmission electron microscopy. Intraoperative electrophysiological routine monitoring was extended by recording paraspinal muscles above and below the laminotomy level. RESULTS: Light microscopy revealed tiny peripheral nerves piercing the pia mater of the FT and entering its fibrous core. Transmission electron microscopy unveiled within the fibrous core of the FT myelinated nerve structures in 8 of the 53 patients and unmyelinated ones in 10 of the 53 patients. Both nerve endings encapsulated in fibrous tissue or unencapsulated nonmyelinated Schwann cell nerve bundles, that is, Remak cells, were found. Those nerve endings resembled mechanoreceptor and nociceptive receptor structures found in human skin, muscle tendons, and skeletal ligaments. Specifically, we found Ruffini mechanoreceptors and in addition nerve endings which resembled nociceptive glioneural structures of the skin. Bipolar electrostimulation of the FT was associated with paraspinal muscle activity above and below the spinal segment at which the FT was stimulated. CONCLUSION: Morphological and electrophysiological results indicate the presence of functional sensory nerve endings in the FT. Like other spine ligaments, the FT may serve as a proprioceptive element but may also contribute to back pain in spine disorders.

This study investigated the prevalence of embryonic and connective tissue elements in the filum terminale (FT) of patients with tethered cord syndrome (TCS), examining both typical and pathological histology. The FT specimens from 288 patients who underwent spinal cord detethering from 2013 to 2021 were analyzed. The histopathological examination involved routine hematoxylin and eosin staining and specific immunohistochemistry when needed. The patient details were extracted from electronic medical records. The study found that 97.6% of the FT specimens had peripheral nerves, and 70.8% had regular ependymal cell linings. Other findings included ependymal cysts and canals, ganglion cells, neuropil, and prominent vascular features. Notably, 41% showed fatty infiltration, and 7.6% had dystrophic calcification. Inflammatory infiltrates, an underreported finding, were observed in 3.8% of the specimens. The research highlights peripheral nerves and ganglion cells as natural components of the FT, with ependymal cell overgrowth and other tissues potentially linked to TCS. Enlarged vessels may suggest venous congestion due to altered FT mechanics. The presence of lymphocytic infiltrations and calcifications provides new insights into structural changes and mechanical stress in the FT, contributing to our understanding of TCS pathology.

OBJECTIVE: While resection of the dural attachment has been shown by Simpson and others to reduce recurrence rates for intracranial meningiomas, the oncological benefit of dural resection for spinal meningiomas is less clear. The authors performed a systematic analysis of the literature, comparing recurrence rates for patients undergoing various Simpson grade resections of spinal meningiomas to better understand the role of dural resection on outcomes after resection of spinal meningiomas.METHODS: The PubMed/Medline database was systematically searched to identify studies describing oncological and clinical outcomes after Simpson grade I, II, III, or IV resections of spinal meningiomas.RESULTS: Thirty-two studies describing the outcomes of 896 patients were included in the analysis. Simpson grade I, grade II, and grade III/IV resections were performed in 27.5%, 64.6%, and 7.9% of cases, respectively. The risk of procedure-related complications (OR 4.75, 95% CI 1.27–17.8, p = 0.021) and new, unexpected postoperative neurological deficits (OR ∞, 95% CI NaN–∞, p = 0.009) were both significantly greater for patients undergoing Simpson grade I resections when compared with those undergoing Simpson grade II resections. Tumor recurrence was seen in 2.8%, 4.1%, and 39.4% of patients undergoing Simpson grade I, grade II, and grade III/IV resections over a mean radiographic follow-up period of 99.3 ± 46.4 months, 95.4 ± 57.1 months, and 82.4 ± 49.3 months, respectively. No significant difference was detected between the recurrence rates for Simpson grade I versus Simpson grade II resections (OR 1.43, 95% CI 0.61–3.39, p = 0.43). A meta-analysis of 7 studies directly comparing recurrence rates for Simpson grade I and II resections demonstrated a trend toward a decreased likelihood of recurrence after Simpson grade I resection when compared with Simpson grade II resection, although this trend did not reach statistical significance (OR 0.56, 95% CI 0.23–1.36, p = 0.20).CONCLUSIONS: The results of this analysis suggest with a low level of confidence that the rates of complications and new, unexpected neurological deficits after Simpson grade I resection of spinal meningiomas are greater than those seen with Simpson grade II resections, and that the recurrence rates for Simpson grade I and grade II resections are equivalent, although additional, long-term studies are needed before reliable conclusions may be drawn.