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Background: Historically, symptomatic, benign intradural extramedullary (IDEM) spine tumors have been managed with surgical resection. However, minimal robust data regarding patient-reported outcomes (PROs) following treatment of symptomatic lesions exists. Moreover, there are increasing reports of radiosurgical management of these lesions without robust health-related quality of life data., Objective: To prospectively analyze PROs among patients with benign IDEM spine tumors undergoing surgical resection to define the symptomatic efficacy of surgery., Methods: Prospective, single-center observational cohort study of patients with benign IDEM spine tumors undergoing open surgical resection. Pre- and postoperative Brief Pain Index (BPI) and MD Anderson Symptom Inventory (MDASI) questionnaires were used to quantitatively assess their symptom control after surgical intervention. Matched pairs were analyzed with the Wilcoxon signed-rank test., Results: A total of 57 patients met inclusion criteria with both pre- and postoperative PROs. There were 35 schwannomas, 18 meningiomas, 2 neurofibromas, 1 paraganglioma, and 1 mixed schwannoma/neurofibroma. Most patients were American Spinal Injury Association Impairment (ASIA) E (93%) with high-grade spinal cord compression (77%), and underwent either a 2 or 3 level laminectomy (84%). Surgical resection resulted in statistically significant improvement in all 3 composite BPI constructs of pain-severity, pain-interference, and overall patient pain experience (P < .0001). Surgical resection resulted in statistically significant improvements in all composite scores for the MDASI core symptom severity, spine tumor, and disease interference constructs (P < .01). Three patients (5%) had postoperative complications requiring surgical interventions (2 wound revisions and 1 ventriculo-peritoneal shunt)., Conclusion: Surgical resection of IDEM spine tumors provides rapid, significant, and durable improvement in PROs., (© Congress of Neurological Surgeons 2021.)

Intramedullary spinal cord tumors (IMSCT) comprise a rare subset of CNS tumors that have distinct management strategies based on histopathology. These tumors often present challenges in regards to optimal timing for surgery, invasiveness, and recurrence. Advances in microsurgical techniques and technological adjuncts have improved extent of resection and outcomes with IMSCT. Furthermore, adjuvant therapies including targeted immunotherapies and image-guided radiation therapy have witnessed rapid development over the past decade, further improving survival for many of these patients. In this review, we provide an overview of types, epidemiology, imaging characteristics, surgical management strategies, and future areas of research for IMSCT., Competing Interests: Disclosure M.H. Bilsky receives royalties from Globus and DePuy/Synthes and is on the Speaker’s Bureau for Brainlab and Varian. The other authors have nothing to disclose., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Despite major advances in radiation and systemic treatments, surgery remains a critical step in the multidisciplinary treatment of metastatic spinal cord tumors., Objective: To describe the indications, rationale, and technique of "hybrid therapy" (separation surgery and concomitant spine stereotactic radiosurgery [SRS]) along with practical nuances., Methods: Separation surgery describes a posterolateral approach for circumferential epidural decompression and stabilization. The goal is to decompress the spinal cord, stabilize the spine, and create adequate separation between the neural elements and the tumor for SRS to achieve durable tumor control., Results: A transpedicular route to achieve ventrolateral access and limited resection of the tumorous vertebral body is carried out. In the setting of high-grade cord compression, caution must be taken when performing the tumor decompression. "Separation" of the ventral epidural tumor component anteriorly creates space for concomitant SRS while a simple laminectomy would not adequately achieve this goal. Dissection of the posterior longitudinal ligament allows maximal ventral decompression. Gross total tumor resection is not crucial for durable tumor control using the "hybrid therapy" model. Thus, attempts at ventral tumor resection may unnecessarily increase operative morbidity. Cement augmentation of the construct or vertebral body may improve construct stability. CT myelogram is the preferred exam for postoperative SRS planning. Radiosurgical planning constitutes a multidisciplinary effort and guidelines for contouring in the postoperative setting have recently become available., Conclusion: Separation surgery is an effective, well-tolerated, and reproducible surgery. It provides safe margins for concomitant SRS. Combined, this "Hybrid Therapy" allows durable local control, maintenance of spinal stability, and palliation of symptoms, while minimizing operative morbidity., (Copyright © 2018 by the Congress of Neurological Surgeons.)

OBJECTIVE Myxopapillary ependymoma is a benign WHO Grade I tumor most commonly located in the conus-cauda equina region of the spine. Although this tumor is defined by an overall excellent survival, dissemination throughout the whole neuraxis occurs frequently. The current study evaluated the clinical characteristics and significance of myxopapillary ependymoma metastases. METHODS Patients who underwent surgery from 2005 to 2015 for treatment of spinal myxopapillary ependymoma were included in the study. Charts were reviewed for primary tumor symptoms and initial treatment, local recurrence, response to salvage therapy, and presence and behavior of distant metastases. RESULTS Nineteen patients with spinal myxopapillary ependymoma were included in the study (52.6% female). The median age at first diagnosis was 32 years old (range 9-58 years old), and 26.3% were ≤ 18 years old. The median follow-up of all included patients was 48 months (range 6-456 months). Of the primary tumors, 84.2% were located in the lower thoracic or upper lumbar spine, spanning 1-3 levels in 94.7%. All patients underwent surgery for initial treatment: in 78.9% a gross-total resection (GTR) was achieved, with adjuvant radiation therapy (RT) in 20%. Of the 21.2% who underwent a subtotal resection (STR), 75% underwent postoperative RT. Tumor progression was noted in 26.3% of patients after a median 36-month follow-up (range 12-240 months). In 57.9% of patients, distant metastases were found, of which 36.4% were present at initial diagnosis. Further metastases occurred within a median of 20 months (range 2-360 months). Following a diagnosis of metastatic tumor, 72.7% did not show progression and no symptoms were observed during a median follow-up of 36 months (range 6-216 months). Metastases occurred in all parts of the neuroaxis, but were principally localized in the thoracic and sacral spine in 38.9% and 33.3%, respectively; the brain was involved in 11.1%. In 54.5%, more than 1 level was affected. Overall survival was 100% with an excellent clinical and neurological outcome in 78.9% of cases. CONCLUSIONS Metastatic dissemination within the CNS can be observed in many patients with myxopapillary ependymoma. GTR of the primary tumor should be the primary treatment goal, and additional RT is recommended after STR. For distant metastases of myxopapillary ependymoma without clinical manifestation, close clinical and MRI follow-up represents a sufficient strategy because most of the metastases remain asymptomatic and do not show progression over time. Additional resection or irradiation as salvage therapy would be recommended if metastases become symptomatic.

Opinion Statement: Intramedullary spinal cord tumors are rare central nervous system tumors with unique challenges due to the eloquence of the surrounding tissue. Their treatment and prognosis is largely dependent on tumor histology and patient functionality. The introduction and advancement of microsurgical techniques have made surgery the mainstay of treatment for intramedullary tumors. Tumors that are well demarcated (e.g., ependymomas, hemangioblastomas) can be resected for cure, while more infiltrative tumors (e.g., high-grade astrocytomas) are typically managed with biopsies or limited resections in order to minimize the significant risk of damage to the spinal cord. The use of more aggressive surgical resection for astrocytoma is controversial but may have an increasing role in select cases. The use of intraoperative neurophysiologic monitoring and intraoperative ultrasound may help guide the extent of surgery while minimizing damage to normal tissue. Advances in MRI technology have greatly aided the diagnosis and preoperative planning of intramedullary tumors. Further advances in intraoperative MRI may make this a useful tool in guiding extent of resection. Preoperative functional status is the most important predictor of neurologic outcome, while histology and extent of resection are the most important predictors of progression-free survival. The use of adjuvant radiation and chemotherapy is dependent on patient age and histology but is largely reserved for high-grade tumor histologies or systemic involvement. Children are particularly at risk of radiation-induced injury, and these cases may benefit from more focused stereotactic radiation where necessary. Further studies are needed to support new surgical strategies minimizing destabilization and to investigate new forms of adjuvant therapy to minimize toxicity.

Background Context: Pathologic vertebral compression fractures (VCFs) represent a major source of morbidity and diminished quality of life in the spinal oncology population. Procedures with low morbidity that effectively treat patients with pathologic fractures are especially important in the cancer population where life expectancy is limited. Vertebroplasty and kyphoplasty are often not effective for mechanically unstable pathologic fractures extending into the pedicle and facet joints. Combination of cement augmentation and percutaneous instrumented stabilization represents a minimally invasive treatment option that does not delay radiation and systemic therapy., Purpose: The objective of the study was to evaluate the safety and efficacy of cement-augmented short-segment percutaneous posterolateral instrumentation for tumor-associated VCF with pedicle and joint involvement., Methods: Forty-four consecutive patients underwent cement-augmented percutaneous spinal fixation for unstable tumors between 2011 and 2014. Retrospective analysis of prospectively collected data, including visual analog pain scale (VAS) response score and procedural complications, was performed., Results: Patients with a median composite Spinal Instability Neoplastic Scale score of 10 (range=8-15) were treated with constructs spanning one to four disk spaces (median of two spaces, constituting 84% of all cases). The proportion of patients with severe pain decreased from 86% preoperatively to 0%; 65% of patients reported no referable instability pain postoperatively. There was one adjacent-level fracture responsive to kyphoplasty, and one case of asymptomatic screw pullout. Two patients subsequently required decompression in the setting of disease progression despite radiation; there was no perioperative morbidity., Conclusions: Percutaneous cement-augmented posterolateral spinal fixation is a safe and effective option for palliation of appropriately selected mechanically unstable VCF that extends into pedicle and/or joint., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: Sterilization of surgical margins for lesions involving the dura is complicated by the tolerance of the spinal cord and/or cauda equina, especially in the setting of prior radiation therapy (RT); use of intraoperative brachytherapy may allow local delivery of therapeutic dose without damaging sensitive structures., Methods and Materials: Patients with malignant lesions involving the dura received intraoperative brachytherapy with a (32)P plaque after maximal resection of the tumor. Local recurrence (LR) was analyzed using competing risks analysis; overall survival was analyzed using Kaplan-Meier statistics., Results: Between September 2009 and April 2013, 68 patients with 69 lesions in the spine were treated with the (32)P plaque. Median followup was 10 months. Most patients (n=59, 85.5%) had previously been treated with at least one course of prior RT to the treated site. About 38 (55%) lesions received postoperative RT (median dose, 30 Gy; range, 18-30 Gy). The LR and overall survival at 12 months were 25.5% (95% confidence interval [CI]=15.5-37%) and 59.5% (95% CI=46-73%), respectively. For patients who received postoperative RT, LR at 12 months was 18.5% (95% CI=7.5-33%) compared with 34% (95% CI=18-51%) for those who were treated with the plaque alone (p=0.08 and 0.04 on univariate and multivariable analysis, respectively). There were no acute or long-term complications from treatment observed in this cohort., Conclusions: The (32)P intraoperative brachytherapy plaque is a useful adjunct to surgical intervention for primary recurrent and metastatic lesions of the spine involving the dura, and is not associated with additional toxicity., (Copyright © 2015 American Brachytherapy Society. Published by Elsevier Inc. All rights reserved.)

Objective: A new paradigm for the radiotherapeutic management of paraspinal tumors has emerged. Intensity-modulated radiotherapy (IMRT) has gained wide acceptance as a way of delivering highly conformal radiation to tumors. IMRT is capable of sparing sensitive structures such as the spinal cord of high-dose radiation even if only several millimeters away from the tumor. Image-guided treatment tools such as cone beam computed tomography coupled with IMRT have reduced treatment errors associated with traditional radiotherapy, making highly accurate and conformal treatment feasible., Methods: This review discusses the physics of image-guided radiotherapy, including immobilization, the radiobiological implications of hypofractionation, as well as outcomes. Image-guided technology has improved the accuracy of IMRT to within 2 mm of error. Thus, the marriage of image guidance with IMRT (IG IMRT) has allowed the safe treatment of spinal tumors to a high dose without increasing the risk of radiation-related toxicity. With the use of near real-time image-guided verification, very-high-dose radiation has been given for tumors in standard fractionation, hypofractionated, and single fraction schedules to doses beyond levels traditionally believed safe in terms of spinal cord tolerance., Results: Clinical results, in terms of treatment-related toxicity and tumor control, have been very favorable. With follow-up periods extending beyond 30 months, tumor control rates with single fraction IG IMRT (1800-2400 cGy) are in excess of 90%, regardless of histology, and without serious sequelae such as radiation myelopathy. Patients also report correspondingly high rates of palliation. Excellent results, both in terms of tumor control and minimal toxicity, have been consistently reported in the literature., Conclusion: IG IMRT represents a significant technological advance. Paraspinal IG IMRT is proof of principle, making it possible to give very-high-dose radiation within close proximity to the spinal cord. By reducing treatment-related uncertainties, margins around tumors can be shortened, thereby reducing the volume of normal tissue that must be irradiated to tumoricidal doses, reducing the likelihood of toxicity. Similarly, higher doses of radiation can be administered safely, improving the likelihood of eradication. Dose escalation can be done to increase the likelihood of tumor cell kill without increasing the dose given to nearby sensitive structures.

The treatment of metastatic spine tumors has evolved significantly over the past 20 years due largely to improvements in magnetic resonance imaging; decompressive surgical techniques, spinal instrumentation, and high-dose conformal radiation. These advances have improved our ability to provide meaningful palliation for patients who often have significant medical and cancer-related issues. Despite technical advances that have improved our ability to maintain neurologic function, stabilize the spine, and prevent local tumor recurrences, significant barriers to rehabilitation remain. This article reviews these primary barriers to rehabilitation of the cancer patient following spine surgery and emphasizes the importance of diagnostic accuracy and a comprehensive team approach to the treatment of pain and other disorders in this population.

Introduction: Considerable controversy exists regarding the appropriate management of spinal involvement in neuroblastoma (NB) patients. We review a large group of such patients and offer treatment recommendations., Methods: Forty-six patients with epidural and/or neural foraminal involvement treated between 1987 and 1998 were staged according to the International NB Staging System (INSS) and classified as high-risk (INSS stage 4; n = 31) or low-risk (INSS stage <4; n = 15). Of 13 high- risk patients with normal neurologic examinations and no radiographic high-grade spinal cord compression (HGSCC), 12 were treated initially with chemotherapy, and only 1 demonstrated neurologic deterioration. HGSCC was present in 18 patients with high-risk NB; 7 of 10 (70%) treated initially with chemotherapy and 6 of 6 (100%) managed initially with operation improved or remained stable. All 9 low-risk patients with normal neurologic examinations and no HGSCC remained neurologically intact following operations (n = 7) or chemotherapy (n = 2). All 4 low-risk patients with HGSCC treated with operations improved or remained stable, and 0 of 2 (0%) low-risk patients treated initially with chemotherapy remained stable. Spinal deformities occurred in 2 of 16 patients (12.5%) treated nonoperatively and in 9 of 30 (30.0%) who underwent operations., Conclusions: High-risk NB patients with spinal involvement but normal neurologic examinations should be offered chemotherapy. High-risk patients with HGSCC may respond to chemotherapy, but a small percentage will require operations for progressive neurologic deficits. Chemotherapy may be avoided in low-risk patients who are offered potentially curative operations. Patients treated with operations for epidural disease are at high risk of subsequently developing spinal deformity., (Copyright 2003 S. Karger AG, Basel)

Reoperation for malignant disease of the cervicothoracic spine can lead to compromised wound healing secondary to poor tissue quality from previous operations, heavily irradiated beds, and concomitant steroid therapy. Other complicating factors include exposed dura and spinal implants. Introducing well-vascularized soft tissue to obliterate dead space is critical to reliable wound healing. The purpose of this study was to determine the efficacy of the trapezius turnover flap in the management of these complex wounds. This study is a retrospective review of all patients undergoing trapezius muscle turnover flaps for closure of complex cervicothoracic wounds after spinal operations for metastatic or primary tumors. Six patients (3 male/3 female) were operated over an 18-month period (mean patient age, 43 years). Primary pathologies included radiation-induced peripheral nerve sheath tumor (N = 2), chondrosarcoma (N = 1), nonsmall-cell lung cancer (N = 1), paraganglioma (N = 1), and spindle cell sarcoma (N = 1). Trapezius muscle turnover flaps were unilateral and based on the transverse cervical artery in every patient. Indication for flap closure included inability to perform primary layered closure (N = 3), open wound with infection (N = 2), and exposed hardware (N = 1). All patients had previous operations of the cervicothoracic spine (mean, 5.8 months; range 2-9 months) for malignant disease and prior radiation therapy. Exposed dura was present in all patients, and 2 patients had dural repairs with bovine pericardial patches. Spinal stabilization hardware was present in 4 patients. All patients underwent perioperative treatment with systemic corticosteroids. All flaps survived, and primary wound healing was achieved in each patient. The only wound complication was a malignant pleural effusion communicating with the back wound, which was controlled with a closed suction drain. All wounds remained healed during the follow-up period. Four patients died from progression of disease within 10 months of surgery. The trapezius turnover flap has been used successfully when local tissue conditions prevent primary closure, or in the setting of open, infected wounds with exposed dura and hardware. The ease of flap elevation and minimal donor site morbidity make it a useful, single-stage reconstructive option in these difficult wounds.

Objective and Importance: Sclerosing epithelioid fibrosarcoma (SEF) is a rare mesenchymal neoplasm composed of rounded, vimentin-immunoreactive tumor cells disposed in nests and cords within a hyalinized collagenous matrix. Most examples arise in the deep skeletal muscles of adults. The cases recorded to date have been characterized by protracted clinical evolutions with a tendency for stubborn local recurrence, followed by late metastasis. Accordingly, SEF has been regarded as a low-grade sarcoma. A single instance of brain and vertebral metastasis has been described. We report three examples of SEF distinguished by primary involvement of the neuraxis at initial presentation., Clinical Presentation: Two tumors had intracranial, calvarial and extracalvarial, soft-tissue components, whereas the third tumor manifested as a paraspinal mass with extension into the T12-L1 neural foramen and invasion of the T12 nerve root., Intervention: All three affected patients experienced local recurrence and distant metastasis after resection of the primary site. These complications appeared early in the disease course in two cases. In no case was there a response to adjuvant chemotherapy or radiotherapy., Conclusion: Our experience indicates that SEFs arising along the neuraxis may demonstrate unexpectedly aggressive clinical behavior, compared with those arising in the more typical location of deep skeletal muscles.