The clinical utility of the Spinal Instability Neoplastic Score (SINS) system in spinal epidural metastases: a retrospective study.

STUDY DESIGN: A retrospective study. OBJECTIVES: This study assessed the clinical utility of the Spinal Instability Neoplastic Score (SINS) in relation to the surgical treatment of spinal epidural metastasis and factors important for surgical decision-making. These factors include epidural spinal cord compression (ESCC), patient prognosis and neurologic status. SETTING: Queen Elizabeth II Health Sciences Centre, Halifax, Canada. METHODS: We identified 285 patients with spinal metastatic disease. Data were extracted through a retrospective review. SINS and ESCC were scored based on CT and MRI, respectively. RESULTS: Patients were grouped into stable (35%), potentially unstable (52%), and unstable (13%) groups. The overall incidence of metastatic spinal deformity was 9%. Surgical interventions were performed in 21% of patients, including decompression and instrumented fusion (70%), decompression alone (17%), percutaneous vertebral augmentation (9%), and instrumented vertebral augmentation (5%). The use of spinal instrumentation was significantly associated with unstable SINS (p = 0.005). Grade 3 ESCC was also significantly associated with unstable SINS (p < 0.001). Kaplan-Meier analysis revealed that SINS was not a predictor of survival (p = 0.98). In the radiotherapy-alone group, a significant proportion of patients with potentially unstable SINS (30%) progressed into unstable SINS category at an average 364 ± 244 days (p < 0.001). CONCLUSION: This study demonstrated that more severe categories of SINS were associated with higher degrees of ESCC, and surgical interventions were more often utilized in this group with more frequent placement of spinal instrumentation. Although SINS did not predict patient prognosis, it correlates with the progression of metastatic instability in patients treated with radiotherapy.

Analysis of seven prognostic scores in patients with surgically treated epidural metastatic spine disease.

BACKGROUND: Prognostic scores have been proposed to guide the treatment of patients with metastatic spine disease (MSD), but their accuracy and usefulness are controversial. The aim of this study was to evaluate seven such prognostic scoring systems. The following prognostic scores were compared: Tomita, Van der Linden (VDL), Bauer modified (BM), Oswestry Spinal Risk Index (OSRI), Tokuhashi original (T90), Tokuhashi revised (TR05), and modified Tokuhashi revised (TR17). METHODS: We retrospectively reviewed all our patients who underwent surgery for spinal metastases, February 2008-January 2015. We classified all 223 patients into the predicted survival-time categories of each of the 7 scoring systems and then tallied how often this was correct vis-à-vis the actual survival time. Accuracy was also assessed using receiver operating characteristic (ROC) analysis at 1, 3, and 12 months. RESULTS: The median (95% CI) survival of the 223 patients was 13.6 (7.9-19.3) months. A groupwise ROC analysis showed sufficient accuracy for 3-month survival only for TR17 (area under the curve [AUC] 0.71) and for 1-year survival for T90 (AUC 0.73), TR05 (AUC 0.76), TR17 (AUC 0.76), Tomita (AUC 0.77), and OSRI (AUC 0.71). A pointwise ROC score analysis showed poor prognostic ability for short-term survival (1 and 3 months) with sufficient accuracy for T90 (AUC 0.71), TR05 (AUC 0.71), TR17 (AUC 0.71), and the Tomita score (AUC 0.77) for 1-year survival. CONCLUSION: The TR17 was the only prognostic system with acceptable performance here. More sophisticated assessment tools are required to keep up with present and future changes in tumor diagnostics and treatment.

Endoscope-assisted anterolateral approach to a recurrent cervical spinal chordoma.

BACKGROUND: The anterolateral approach (ALA) enables to access the craniovertebral junction (CVJ), lower and middle clivus, jugular foramen, and cervical spine from a lateral perspective. It is particularly indicated when dealing with extradural bone tumors. Other rare indications are represented by spondylotic myeloradiculopathy and vascular diseases. METHOD: We describe here the steps to safely perform an anterolateral approach along with a brief description of its indications and limits. CONCLUSION: ALA represents a valid option to treat cervical spine and CVJ bone tumors such as chordomas. Its knowledge can improve the process of approach selection when dealing with such complex cases.

Clinical features and long-term surgical outcomes of pure spinal epidural cavernous hemangioma-report of 23 cases.

BACKGROUND: Pure spinal epidural cavernous malformation (CM) is a rare hypervascular disease that is easily misinterpreted as other imaging-similar epidural lesions. The demographic characteristics, therapeutic strategies, and surgical outcomes associated with this vascular entity remains unclear. METHODS: A retrospective review of patients with pathologically proven, pure epidural CM from 2001 to 2018 was conducted. All data that included clinical manifestations, radiographic features, and treatment modalities were analyzed. RESULTS: Twenty-three consecutive patients with an average age of 51.5 ± 8.4 years old (range 38-70 years old) were included; of these, 52.2% were female patients. Clinical manifestation included chronic progressive nerve root disturbance syndrome in 7 patients (30.4%) and myelopathy in 16 patients (69.6%). The CM level was predominately thoracic (n = 14, 60.9%) or lumbar (n = 6, 26.1%), with the dorsal epidural space (n = 14, 60.9%) the most common site. The initial clinical diagnoses were schwannoma (n = 11, 52.2%), meningioma (n = 5, 21.7%), angioma (n = 3, 13.1%), recurrent CMs (n = 2, 8.7%), and metastatic tumor (n = 1, 4.3%). Fifteen lesions (65.2%) were isointense on T1-weighted images, and all lesions were hyperintense on T2-weighted images, with homogenously strong enhancement observed in 17 lesions (73.9%). Total resection was achieved in 18 patients (78.3%) and usually resulted in excellent clinical outcomes (n = 21, 91.3%). No patients experienced recurrence of symptoms, and lesion relapse during follow-up. CONCLUSION: Total surgical removal of epidural CM can usually achieve satisfactory outcomes in patients with a chronic clinical course and should be recommended. Subtotal removal of tumors can also benefit patients, and guaranteed a long recurrent free time after surgery. A good preoperative neurological condition usually leads to good outcomes.

MRI-Guided Cryoablation of Epidural Malignancies in the Spinal Canal Resulting in Neural Decompression and Regrowth of Bone.

OBJECTIVE: The purpose of this article is to describe the use of MRI to safely monitor cryoablation for the treatment of spinal epidural malignancies. CONCLUSION: Use of MRI guidance to monitor percutaneous cryoablation allows ablation margins more distinct than those allowed by heat-based ablation modalities. MRI-guided cryoablation is a feasible option for treating epidural tumors involving the spinal canal, resulting in successful decompression of the tumor away from the spinal cord with regrowth of previously eroded bone around the spinal canal.

Enlarging teratoma syndrome.

INTRODUCTION: Teratomas are derived from all three germ layers and make up 3% of all childhood tumors. They are histologically classified as mature or immature. We present a case that was operated on when 30 days old for a sacrococcygeal mature teratoma and then showed long segment involvement in the thoracolumbar region 9 months after the surgery. The MRI (magnetic resonance imaging) showed a mass starting at the thoracal 4 level and extending to the lumbar 3 level with significant spinal cord compression in the extradural space. RESULT: The laminae between thoracal 4 and lumbar 3 levels were removed en bloc at a single surgical session and laminoplasty was performed after tumor resection. We also removed the tumor growing into the extrapleural space at the thoracal 5, 6, and 9, 10, 11, 12 levels using the costotransversectomy procedure. CONCLUSIONS: We emphasize with this case that mature teratomas can show aggressive growth following surgery and that the development of spinal deformities can be prevented with laminoplasty.

Predictive factors of survival in a surgical series of metastatic epidural spinal cord compression and complete external validation of 8 multivariate models of survival in a prospective North American multicenter study.

BACKGROUND: This study was designed to identify preoperative predictors of survival in surgically treated patients with metastatic epidural spinal cord compression (MESCC), to examine how these predictors are related to 8 prognostic models, and to perform the first full external validation of these models in accordance with the Transparent Reporting of a Multivariable Prediction Model for Individual Prognosis or Diagnosis (TRIPOD) statement. METHODS: One hundred forty-two surgically treated patients with MESCC were enrolled in a prospective, multicenter North American cohort study and were followed for 12 months or until death. Cox regression was used. Noncollinear predictors with < 10% missing data, with ≥ 10 events per stratum, and with P < .05 in a univariate analysis were tested through a backward stepwise selection. For the original and revised Tokuhashi prognostic scoring systems (PSSs), Tomita PSS, modified Bauer PSS, van der Linden PSS, Bartels model, Oswestry Spinal Risk Index, and Bollen PSS, this study examined calibration graphically, discrimination with Harrell c-statistics, and survival stratified by risk groups with the Kaplan-Meier method and log-rank test. RESULTS: The following were significant in the univariate analysis: type of primary tumor, sex, organ metastasis, body mass index, preoperative radiotherapy to MESCC, physical component (PC) of the 36-Item Short Form Health Survey, version 2 (SF-36v2), and EuroQol 5-Dimension (EQ-5D) Questionnaire. Breast, prostate and thyroid primary tumor (HR: 2.9; P =.0005), presence of organ metastasis (hazard ratio (HR): 2.0; P = .005) and SF-36v2 PC (HR: 0.95; P < .0001) were associated with survival in multivariable analysis. Predicted prognoses poorly matched observed values on calibration plots; Bartels model calibration slope was 0.45. Bollen PSS (0.61; 95% CI: 0.58-0.64) and Bartels model (0.68; 95% CI: 0.65-0.71) had the lowest and highest c-statistics, respectively. CONCLUSIONS: The primary tumor type (breast, prostate, or thyroid), an absence of organ metastasis, and a lower degree of physical disability are preoperative predictors of longer survival for surgical MESCC patients. These results are in keeping with current models. This full external validation of 8 prognostic PSSs or model of survival in surgical MESCC patients has revealed that calibration is poor, especially for long-term survivors, whereas discrimination is possibly helpful.

Purely Spinal Epidural Capillary Hemangiomas.

Capillary hemangiomas (CAs) are benign endothelial cell neoplasms that are often encountered superficially in the soft tissues of the head and neck region. Most of the reported purely spinal epidural hemangiomas have been of cavernous type, and the occurrence of purely spinal epidural CA is exceedingly rare, and there are only 12 reported cases of spinal epidural CAs in the English literature. Herein, the authors report the 13th case of purely spinal epidural CAs, and the clinical characteristics, histopathological features, and treatment were also investigated.

SPINAL DUMBBELL EPIDURAL HEMANGIOMA: TWO STAGE/SAME SITTING/SAME POSITION POSTERIOR MICROSURGICAL AND TRANSTHORACIC ENDOSCOPIC RESECTION - CASE REPORT AND REVIEW OF THE LITERATURE.

- Spinal dumbbell tumors are defined by a narrowing at the point where they penetrate the intervertebral foramina or dura mater, assuming an hourglass or dumbbell shape. Dumbbell-shaped spinal hemangiomas are extremely rare. We describe a dumbbell spinal tumor (epidural cavernous hemangioma) resected by a 2-stage single-sitting combined approach. We also conduct a substantial literature review of the subject. We present a case of a 78-year-old male who was found to have a homogeneously enhancing, dumbbell-shaped, intraspinal, extradural tumor mass extending into the left chest cavity. The tumor was resected with a single-sitting 2-stage posterior technique: a microsurgical approach, followed by endoscopic resection via a thoracoscopic approach. There are several reports in the literature on the combined approach for dumbbell tumors of the spinal cord. Our case is the first to describe 2-stage combined surgery in 1 sitting for dumbbell hemangioma with the patient in the lateral decubitus position for the thoracoscopic part of the surgery; and the use of a fat pad, which was applied in the neuroforamen via the posterior route, as a marker for resection during the transthoracic procedure.

Impact of spinal cord compression from intradural and epidural spinal tumors on perioperative symptoms-implications for surgical decision making.

Spinal cord or cauda equina compression (SCC) is an increasing challenge in clinical oncology due to a higher prevalence of long-term cancer survivors. Our aim was to determine the clinical relevance of SCC regarding patient outcome depending on different tumor entities and their anatomical localization (extradural/intradural/intramedullary). We retrospectively analyzed 230 patients surgically treated for SCC. Preoperative status for pain and neurological impairment were correlated to the degree of compression, tumor location, and early as well as short-term follow-up outcome parameters. Interestingly, we did not observe any differences between intradural-extramedullary compared to extradural tumors. Unilaterally localized tumors were likely to present with pain (72.9 %, p < 0.01), whereas concentric growth was associated with motor deficits (41.0 %, p < 0.01, as primary symptom, 49.3 % on admission, p < 0.05). In concentric tumors, the pain pattern was diffuse (40.5 % vs. 17.5 in unilateral disease, p < 0.01), whereas unilateral tumors resulted in localized pain (61.4 % local axial or radicular, p < 0.01). Diffuse pain, patients without a sensory or motor deficit, progressive disease, cervical localization, and a higher degree of stenosis were identified as beneficial for an early improvement in pain (p < 0.05). Notably, 29 % of patients with unchanged pain and 30.8 % with unchanged neurologic function at day 7 postoperative improved during follow-up (p < 0.001). Our data demonstrate that the preoperative tumor anatomy in patients with SCC was closely related to their presenting symptoms and early clinical outcome. The detailed analysis elucidates the biology of SCC and might thereby aid in determining which patients will benefit from surgery.

Monitoring rate and predictability of intraoperative monitoring in patients with intradural extramedullary and epidural metastatic spinal tumors.

STUDY DESIGN: Single-center retrospective study. OBJECTIVES: To evaluate the monitoring rate, sensitivity and specificity of intraoperative monitoring (IOM) during removal of intradural extramedullary (IDEM) or epidural metastatic spinal tumors. Also, to assess the efficacy of monitoring somatosensory-evoked potentials (SSEP) when motor-evoked potentials (MEP) are not measurable. SETTING: The Neuro-Oncology Clinic, National Cancer Center, Korea. METHODS: Patients (n=101) with IDEM or epidural metastatic spinal tumors at the cord level underwent surgeries monitored with SSEP and/or MEP. The monitoring rate was defined as negative when MEP or SSEP could not be measured after reversal of the neuromuscular block under general anesthesia. Positive IOM changes included more than a 50% change in the MEP or SSEP amplitude and more than a 10% delay in SSEP latency. RESULTS: MEP was measurable in 73% of patients. The MEP monitoring rate in patients with motor power grades of 3 or less was 39%, which was lower than that of SSEP (83%). The sensitivity, specificity and predictability of MEP for motor changes were 93, 90 and 91%, respectively. Conversely, the sensitivity, specificity and predictability of SSEP were 62, 97 and 89%, respectively. In patients in whom MEP was not measurable (n=24), SSEP was monitored with a predictability of 83%. CONCLUSION: In cases of extramedullary spinal tumors, MEP shows a higher sensitivity than SSEP does. However, the monitoring rate of MEP in non-ambulatory patients was lower than that of SSEP. In those cases, SSEP can be useful to monitor for postoperative neurological deficits.

Spinal dumbbell lipoblastoma: a case-based update.

INTRODUCTION: Lipoblastomas are benign tumors that arise from the proliferation of fetal adipose tissue. They are frequently found in the extremities, in the head, and in the neck. Dumbbell conformation (which refers to epidural tumors with extraspinal extension) has been described in only seven lipoblastoma cases so far. CASE REPORT: We describe an 11-month-old patient with a large mediastinal dumbbell lipoblastoma. The mass was excised, but it recurred 1 year later when the MRI showed also multiple pleural lipoblastomatous lesions, thus establishing the diagnosis of lipoblastomatosis. The tumor has been removed with a second surgical procedure. CONCLUSION: Radical excision of lipoblastomas is the gold standard but is not always possible to achieve. Residual lesions can be managed conservatively, on the basis of the benign nature of the tumor and of its potential maturation to normal fat. Recurrence of lipoblastoma appears in 15 % of non-radical excisions but has not yet been described in the form of lipoblastomatosis. A second surgery is indicated if the tumor mass reaches large dimensions, if it increases in size, and if it threatens the functionality of vital organs such as the spinal cord.

Minimally invasive resection of extradural dumbbell tumors of thoracic spine: surgical techniques and literature review.

PURPOSE: Dumbbell-shaped thoracic tumors represent a distinct type of tumor and involve in both the spinal canal and the posterior thoracic cavity. Successful treatment for the tumors depends on gross total resection (GTR) via an open laminectomy and facetectomy or transthoracic transpleural approach. In this case series, we report our experiments with minimally invasive method for the removal of extradural dumbbell thoracic tumor and present related literature review. METHODS: We retrospectively reviewed two patients with dumbbell-shaped thoracic tumors who underwent minimally invasive resection and unilateral transforaminal thoracic intervertebral fusion (TTIF) through unilateral paraspinal muscle approachwith a spotlight expandable tubular retractor. Clinical data, tumor characteristics, and outcomes were analyzed. RESULTS: Two patients underwent successful minimally invasive treatment of their spinal neoplasms. There were no procedure-related complications. The efficacy in terms of neurological recovery, pain improvement and operative variables (length of incision, operative duration, blood loss, and hospital stay) was better when compared with prior published studies. Postoperative CT image demonstrated complete resection of dumbbell tumor in the patients. The solid fusion was obtained after 3 months follow-up and there was no failure of internal fixation. CONCLUSION: If the medial border of intracanal component of extradural dumbbell tumor is near the midline of canal and the pedicles of adjacent vertebrae to tumor are intact, minimally invasive resection of tumor through unilateral paraspinal muscle approach combined with unilateral TTIF is good choice.

Management of infiltrating spinal epidural angiolipoma.

Angiolipomas of the spine are rare benign tumors commonly presenting with compressive myelopathy. The present report describes a case of spinal angiolipoma with thoracic mediastinal extension in a 50-year-old woman. She presented with a long-standing history of mid-back pain with progressive lower extremities weakness. An MRI showed a heterogeneously enhancing mass located in the posterior epidural space of the thoracic spine with mediastinal extension. Histopathological examination demonstrated features consistent with spinal angiolipoma. This report emphasizes the diagnosis and therapeutic management options of infiltrating spinal angiolipomas.

Primary epidural hemangiopericytoma in the sacrum: a rare case and literature review.

Hemangiopericytoma (HPC) is an uncommon highly vascular neoplasm that originated from Zimmerman's pericytes which surrounds the endothelial tissue. Primary epidural HPC of the sacrum is extremely rare. We reported an unusual case of primary epidural malignant HPC of the sacrum that invaded vertebral bone and caused rectum compression in a 57-year-old male for the first time. The patient presented progressive low back pain and ribbon-like stool over 3 months. The surgical intervention involved sacrectomy and en bloc resection of the tumor. We described the clinical, radiological, and histological features of this tumor and reviewed the literature.

Ewing sarcoma of the thoracic epidural space in a young child.

PURPOSE: To report on the clinical course and treatment of Ewing sarcoma of the thoracic epidural space in a 5-year-old girl. METHODS: We present the case of a 5-year-old girl who experienced back pain (day 1); on day 10, the pain had exacerbated and involuntary movements in the lower limbs occurred, and an MRI performed in her local hospital revealed a tumor lesion at the upper thoracic level. RESULTS: On day 13, emergency surgery was performed for partial resection of the tumor. Pathological examination of the resected tumor by immunostaining and gene testing revealed that it was MIC2 positive and an EWS-FLI 1 chimera, respectively, and Ewing sarcoma was diagnosed. The involuntary movements resolved immediately after the surgery. Three weeks after the operation, chemotherapy and radiation therapy were commenced. After 5 months, deep tendon reflexes recovered to normal. MRI showed that the tumor has not recurred at 29 months after surgery. CONCLUSIONS: The majority of epidural patients undergo emergency surgery only after symptom exacerbation, which includes the development of neurological deficits. Thus, preoperative diagnosis of Ewing sarcoma of the epidural space is difficult and diagnosis is frequently made by a post-operative gene test. The resection area is limited to the intralesional margin area because a larger resection is difficult due to the characteristics of the affected region; thus, there is a higher possibility of recurrence and careful follow-up of the case is necessary.

Risk factors for survival of 106 surgically treated patients with symptomatic spinal epidural metastases.

PURPOSE: Evaluation of risk factors for survival in patients surgically treated for symptomatic spinal epidural metastases (SEM). METHODS: One hundred and six patients who were surgically treated for symptomatic SEM in a 10-year period in two cooperatively working hospitals were retrospectively studied for nine risk factors: age, gender, site of the primary tumor, location of the symptomatic spinal metastasis, functional and neurologic status, the presence of visceral metastases and the presence of other spinal and extraspinal bone metastases. Analysis was performed using the Kaplan-Meier method, univariate log-rank tests and Cox-regression models. RESULTS: Overall median survival was 10.7 months (0.2-107.5 months). Overall 30-day complication rate was 33 %. Multivariate Cox-regression analysis showed that fast growing primary tumors (HR 3.1, 95 % CI 1.6-6.2, p = 0.001), the presence of visceral metastases (HR 1.7, 95 % CI 1.0-2.9, p = 0.033) and a low performance status (HR 2.7, 95 % CI 1.1-6.6, p = 0.025) negatively influenced the survival. CONCLUSION: Primary tumor type, presence of visceral metastases and performance status are significant predictors for survival after surgery for symptomatic SEM and should be evaluated before deciding on the extent of treatment. More accurate prediction models are needed to select the best treatment option for the individual patient.

Primary spinal epidural rhabdomyosarcoma: a case report and review of the literature.

BACKGROUND: Primary intraspinal sarcomas are very rare in children. Rhabdomyosarcoma (RMS) is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space. METHOD: We report a 13-year-old girl who presented with back pain, progressive paraparesis, and urinary retention. She had muscular weakness in her lower extremities and absent deep tendon reflex. An epidural dumbbell-shape mass at T11-T12 level was observed on MRI. The patient underwent T12 hemilaminectomy, partial T11 hemilaminectomy, and right facetectomy. A large, firm, dark-red vascular epidural tumor was found compressing the cord. The tumor which extended to intracanal and foraminal parts was removed completely. DISCUSSION: Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.

Pure spinal epidural cavernous hemangioma.

BACKGROUND: Pure epidural cavernous hemangiomas without bony involvement are rare, representing 4% of all spinal epidural tumors. Most of these are case reports and are easily misdiagnosed. METHODS: Herein nine patients (male:female, 5:4, average age: 51 years) with symptomatic pure epidural spinal cavernous hemangioma between 2005 and 2011 were treated, and the clinical, radiological, and pathological records, treatment, and prognosis were discussed. RESULTS: All patients experienced a slowing progressive clinical course, except for one with intralesional hemorrhage. Clinical manifestations included back or radiating pain, sensorimotor deficits, and sphincters disturbance. Eight lesions were isointense on T1- and hyperintense on T2-weighted images with homogenously strongly enhancement and one was mixed signal with heterogeneous enhancement because of intratumoral hemorrhage. Hemilaminotomoy or laminotomy was performed and total resection was achieved. All patients experienced a gradual neurological improvement with no recurrence. CONCLUSIONS: Spinal epidural cavernous hemangioma is a benign vascular malformation that should be excluded in the diagnosis of epidural lesion. Total surgical resection is recommended and usually results in a good prognosis.