Pure epidural extraosseous cavernous hemangioma with thoracic myelopathy: case report and review of literature.

INTRODUCTION: Pure epidural spinal cavernous hemangiomas are rare, benign vascular tumors that account for approximately 4% of all spinal epidural tumors. Due to their dumbbell shape and propensity for foraminal invasion, they are often misdiagnosed and inadequately treated. We present a case of a 58-year-old male with extra-osseous cavernous hemangioma to better aid in diagnosis and management of these lesions. CASE PRESENTATION: A 58-year-old male presented with chronic lower back pain, progressive lower extremity weakness, T10 sensory level, absent lower extremity proprioception, hyperreflexia, and an episode of bowel incontinence. Imaging demonstrated T7-T10 homogenous dorsal epidural mass causing cord signal change. He underwent resection with histopathologic exam revealing a pure epidural cavernous hemangioma. CONCLUSION: Spinal epidural cavernous hemangiomas are exceedingly rare lesions that are often misdiagnosed as nerve sheath tumors and meningiomas. Common features include chronic pain and myelopathy as well as T1 isodensity, T2 hyperintensity, and homogenous enhancement. Uniquely, they present as a lobulated, spindled shape with tapered ends in the dorsal epidural space. Both gross and subtotal resection result in favorable neurologic outcomes.

Epidural angioleiomyoma: an extraordinary cause of compressive myelopathy-MRI findings with histopathological correlation.

BACKGROUND: Angioleiomyomas are benign mesenchymal tumors usually located in the limbs, with anecdotal reports in the spine. We present an atypical case of an epidural spine angioleiomyoma presenting with compressive myelopathy symptoms. The diagnosis was suggested based on MRI findings, and subsequently confirmed histopathologically. RESULTS: This is the first known occurrence of pure spinal epidural angioleiomyoma as a source of compressive myelopathy. The imaging presentation, especially the 'dark reticular sign' on MRI, was crucial in suggesting the diagnosis despite the atypical location CONCLUSION: This report serves to raise awareness among clinicians and radiologists about including angioleiomyoma in differential diagnoses for spinal epidural lesions with indicative MRI features. The favorable outcome after surgical intervention underscores the necessity of swift and accurate diagnosis followed by appropriate treatment for such uncommon spinal tumors.

History of Evolution of Posterolateral Approaches to the Thoracic Spine: From Cure of Pott's Disease to Epidural Tumor Resection.

Since the end of the nineteenth century, the wide dissemination of Pott's disease has ignited debates about which should be the ideal route to perform ventrolateral decompression of the dorsal spine in case of paraplegia due to spinal cord compression in tuberculosis spondylitis. It was immediately clear that the optimal approach should be the one minimizing the surgical manipulation on both neural and extraneural structures while optimizing the exposure and surgical maneuverability on the target area. The first attempt was reported by Victor Auguste Menard in 1894, who described, for the first time, a completely different route from traditional laminectomy, called costotransversectomy. The technique was conceived to drain tubercular paravertebral abscesses causing paraplegia without manipulating the spinal cord. Over the following decades, many other routes have been described all over the world, thus demonstrating the wide interest on the topic. Surgical development has been marked by the new technical achievements and by instrumental/technological advancements, until the advent of portal surgery and endoscopy-assisted techniques. In this article, the authors retraced the milestones of this history up to the present days, through a systematic review on the topic.

Diagnostic value of MRI in coexistence of schwannoma and meningioma mimicking a single dumbbell-shaped tumor in high cervical level. Case series and literature review.

CONTEXT: Concurrent schwannoma and meningioma arising in the high cervical level mimicking a single dumbbell-shaped tumor is significantly rare, most of them were found during the surgeries or postoperative histological findings unexpectedly. The specific feature of schwannoma and meningioma coexistence in high cervical level on MR images has not been clearly described yet. FINDINGS: We presented four cases of concurrent extradural schwannoma and intradural meningioma mimicking a single dumbbell-shaped tumor arising in the high cervical level. There was no interconnection between intradural and extradural masses in any case. In MRI reviews, the signal intensity between intradural lesions and spinal cord was similar on T2 weighted MR images. However, on contrast-enhanced MR images, the intradural lesions were more enhanced than spinal cord and presented as crescent-shaped intradural minor lesions adjacent to the more significantly enhanced extradural major tumor. These MRI findings could not be easily identified without meticulous observation preoperatively. Postoperative pathological findings confirmed the discrete tumors arising in the same cervical level. CONCLUSION: The comparison of signal intensity changes among the spinal cord, intradural tumor and extradural tumor between T2 weighted and contrast-enhanced MR images may be helpful to predict coexistent schwannoma and meningioma in the high cervical level preoperatively. Intradural exploration is highly recommended when less enhanced crescent-shaped intradural minor lesion was observed adjacent to the significantly enhanced dumbbell-shaped major tumor in preoperative MRI findings.

Pure extraosseous spinal epidural cavernous hemangioma presenting with acute paraplegia: a case report.

INTRODUCTION: Spinal hemangiomas are benign vascular tumors that most commonly originate from the osseous structures of the spinal column. Epidural spinal hemangiomas without osseous involvement are uncommon and are classified as pure epidural spinal hemangiomas. Extraosseous spinal epidural cavernous hemangiomas are rarely described and among available reports; most patients present with slowly progressive neurological symptoms. Herein, we present a novel case of acute neurological dysfunction from a pure spinal epidural hemangioma that was managed through surgical resection with good neurological recovery at follow-up. CASE PRESENTATION: A 45-year-old previously healthy man presented to the emergency room with sudden inability to ambulate and was found to have bilateral lower extremity weakness. Magnetic resonance imaging of the spine demonstrated an epidural mass extending out of the right T5/6 neural foramen. The mass enhanced heterogeneously, and the preoperative diagnosis favored an atypical schwannoma. The lesion was surgically removed en-bloc through a midline posterior decompression with instrumentation. Histopathologic examination confirmed cavernous hemangioma pathology. Within 6 weeks of the surgical intervention, the patient had regained full sensorimotor function and these effects were durable through long term follow-up. DISCUSSION: Pure spinal epidural hemangiomas are rare and generally have an insidious clinical course. This case report highlights that these uncommon lesions may present with substantial and acute neurological dysfunction requiring urgent neurosurgical intervention. This should prompt clinicians to consider cavernous hemangioma in the differential diagnosis of patients presenting with acute neurological deterioration and an epidural spinal tumor.

A pilot study of stereotactic boost for malignant epidural spinal cord compression: clinical significance and initial dosimetric evaluation.

PURPOSE: Metastatic epidural spinal cord compression (MESCC) is a devastating complication of advanced malignancy, which can result in neurologic complications and significant deterioration in overall function and quality of life. Most patients are not candidates for optimal surgical decompression and as a result, receive urgent 3D conformal radiotherapy (3DCRT) to prevent or attempt to reverse neurologic progression. Multiple trials indicate that response and ambulatory rates after 3DCRT are inferior to surgery. The advent of stereotactic body radiation therapy (SBRT) has created a method with which a "radiosurgical decompression" boost may facilitate improve outcomes for MESCC patients. METHODS: We are conducting a pilot study to investigate SBRT boost after urgent 3D CRT for patients with MESCC. The aim of the study is to establish feasibility of this two-phase treatment regimen, and secondarily to characterize post-treatment ambulation status, motor response, pain control, quality of life and survival. DISCUSSION: We describe the study protocol and present a case report of one patient. A quality assurance review was conducted after the first seven patients, and resultant dose-constraints were revised to improve safety and feasibility of planning through more conservative organ at risk constraints. There have been no severe adverse events (grade 3-5) to date. We have illustrated clinical and dosimetric data of an example case, where a patient regained full strength and ambulatory capacity. CONCLUSIONS: Our study aims to determine if SBRT is a feasible option in addition to standard 3DCRT for MESCC patients, with the goal to consider future randomized trials if successful. Having a robust quality assurance process in this study ensures translatability going forward if future trials with multicenter and increased patient representation are to be considered. TRIAL REGISTRATION: clinicaltrials.gov; registration no. NCT03529708; https://clinicaltrials.gov/ct2/show/NCT03529708 ; First posted May 18, 2018.

A movement disorder specialist gets a taste of his own medicine.

We present a case in which a movement disorder neurologist developed two different hyperkinetic movements due to corticospinal tract hyperexcitability, which resolved completely with surgical removal of an epidural spinal canal tumor. The first-hand description of these movements creates a unique opportunity for enhanced insight into these complex movement phenomena.

Outcome of Epidural Hematoma: Lessons from Solitary Fibrous Tumor/Hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is rare. In this report, a case of epidural hematoma (EDH) that eventually evolved into SFT/HPC is presented. We describe the possible association between the 2 diseases, which has not been previously reported. CASE DESCRIPTION: A 40-year-old man suffered from an EDH in the right parietal area 12 years ago and accepted conservative treatment. Follow-up computed tomography (CT) scan shows that the density of the right EDH gradually changed from uniform slightly lower density to mixed density. A new CT scan revealed an epidural mass extending to the subcutaneous with local bone destruction. An operation was performed via a large right parietal craniotomy, and the final diagnosis was World Health Organization grade III SFT/HPC after histopathologic examination and immunohistochemical verification. The patient died of deterioration of brain disease 3 months after the final diagnosis. CONCLUSIONS: To our knowledge, this is the first report that HPC occurred in the epidural cavity. We are the first time to describe the possible association between EDH and HPC.

Primary Extraosseous Ewing Sarcoma of the Thoracic Spine Presenting as Chest Pain Mimicking Spinal Schwannoma.

BACKGROUND: Extraosseous Ewing sarcoma (EES) usually has a pseudocapsule and high vascularity, making it well circumscribed and focally dense with contrast enhancement on magnetic resonance imaging (MRI). Consequently, it is difficult to diagnose and distinguish from other spinal tumors, based on pretreatment radiologic findings alone. Here, we present a case of EES involving the thoracic spinal column, which was suspected to be spinal schwannoma through pretreatment radiologic findings. CASE DESCRIPTION: A 54-year-old woman was admitted to our hospital with upper back and left-sided chest pain. Contrast-enhanced MRI of the thoracolumbar spine showed a 17- × 12-mm-sized mass in the epidural region and left neural foramen at the T6-7 level. Our preliminary diagnosis was spinal schwannoma. The patient underwent T6 hemilaminectomy. Intraoperatively, the lesion appeared as gray-colored soft mass with high vascularity, which seemed to have originated from the left T6 nerve root. The tumor was excised with en bloc resection. Histopathologic examination of the lesion revealed classical Ewing sarcoma with high cellularity of small round cells. Immunohistochemistry revealed strong positivity for cluster of differentiation 99 and FLI-1. Intensity-modulated radiation therapy was performed. The patient did not receive chemotherapy. Five years after surgery, follow-up spinal MRI and positron emission tomography computed tomography scan revealed no recurrence of the tumor or new lesions. CONCLUSIONS: Clinicians should consider EES in the differential diagnosis of other neural foraminal spinal tumors, such as schwannoma. If clinicians are confident that EES has been removed completely and there are no other lesions, radiotherapy is sufficient and additional chemotherapy may not be necessary.

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.

Cervical-Thoracic Epidural Giant Cell Reparative Granuloma: Case Report and Review of the Literature.

BACKGROUND: Giant cell reparative granuloma (GCRG) is a rare benign tumor. The jawbone is the most common site of occurrence, followed by sphenoid bone, craniofacial bone, hand and foot bones. The etiology of GCRG is unknown but may be related to an intraosseous hemorrhage following trauma. Despite its benign nature, it could be locally aggressive. To our knowledge, no spinal epidural GCRG case has been reported. CASE DESCRIPTION: A case of man aged 32 years who presented with upper right limb numbness and weakness. Computed tomography showed a round soft tissue mass in the spinal canal at the C7-T1 level. The mass showed isointensity on T1-weighted images, hypointensity on T2-weighted images, and significant enhancement on postcontrast T1-weighted images. The mass localized in the epidural space and was surgically resected. The histologic diagnosis was consistent with GCRG. CONCLUSIONS: Spinal epidural GCRG is rare and is hardly considered in the differential diagnosis. Preoperative diagnosis of GCRG is challenging, and the definitive diagnosis could only be made by pathological examination. Surgical resection is probably an effective therapy for relief of symptoms.

Hematolymphoid Malignancies Presenting with Spinal Epidural Mass and Spinal Cord Compression: A Case Series with Rare Entities.

The epidural space is an uncommon site for involvement by hematolymphoid malignancies, and may present unexpectedly with neurological symptoms related to spinal cord compression. Our objective was to review the clinical and pathologic features of cases with initial presentations of cord compression, subsequently diagnosed as a hematolymphoid malignancy after pathologic examination. Review of the Department of Pathology's archives revealed 15 patients who presented with spinal cord compression due to epidural hematolymphoid malignancies between 2008-2019. These cases involved five primary epidural lymphomas, including an ALK-negative anaplastic large T-cell lymphoma previously not reported at this site, three diffuse large B cell lymphomas, one B-lymphoblastic lymphoma, four cases of myeloid sarcoma, one case with a previous history of acute myeloid leukemia, five cases with plasma cell neoplasms and epidural lesions as the initial presentation of plasma cell myeloma, one case showing aberrant T-cell marker expression, and one case being a histiocytic sarcoma that is rarely reported in the spine. A hematolymphoid malignancy was suspected clinically or radiologically in only five of these cases. These cases represent the spectrum of hematolymphoid malignancies that can involve the epidural space and present for the first time with cord compression, resulting in clinical, radiological and pathologic diagnostic challenges. Their diagnoses require a high degree of awareness, suspicion, and thorough histologic evaluation with ancillary studies for appropriate disease classification and therapeutic intervention. To our knowledge, this is one of the largest and most diverse of such series in the English language literature.

Pure Lumbar Epidural Cavernous Hemangioma with Contralateral Symptoms Complicated by Spondylolisthesis.

Pure spinal epidural cavernous hemangioma with contralateral symptoms is rarely reported. It will be very difficult to diagnose when it is complicated by contralateral symptoms. A 56-year female presented with pure lumbar epidural cavernous hemangioma with contralateral symptoms. Physical examination and imaging including X-ray, CT and MRI, suggested that it is a hemangioma complicated by spondylolisthesis. Laminectomy and exploration was performed. Postoperative X-ray showed satisfactory interbody fusion and reduction of spondylolisthesis. The patient was fully recovered four months postoperatively. Histopathological examination showed that the mass was epidural cavernous hemangioma.

The impact of surgical timing on neurological outcomes and survival in patients with complete paralysis caused by spinal tumours: evaluation of surgery on patients with complete paralysis due to neoplastic epidural spinal cord compression.

AIMS: The aim of this study was to explore the prognostic factors for postoperative neurological recovery and survival in patients with complete paralysis due to neoplastic epidural spinal cord compression. PATIENTS AND METHODS: The medical records of 135 patients with complete paralysis due to neoplastic cord compression were retrospectively reviewed. Potential factors including the timing of surgery, muscular tone, and tumour characteristics were analyzed in relation to neurological recovery using logistical regression analysis. The association between neurological recovery and survival was analyzed using a Cox model. A nomogram was formulated to predict recovery. RESULTS: A total of 52 patients (38.5%) achieved American Spinal Injury Association Impairment Scale (AIS) D or E recovery postoperatively. The timing of surgery (p = 0.003) was found to be significant in univariate analysis. In multivariate analysis, surgery within one week was associated with better neurological recovery than surgery within three weeks (p = 0.002), with a trend towards being associated with a better neurological recovery than surgery within one to two weeks (p = 0.597) and two to three weeks (p = 0.055). Age (p = 0.039) and muscle tone (p = 0.018) were also significant predictors. In Cox regression analysis, good neurological recovery (p = 0.004), benign tumours (p = 0.039), and primary tumours (p = 0.005) were associated with longer survival. Calibration graphs showed that the nomogram did well with an ideal model. The bootstrap-corrected C-index for neurological recovery was 0.72. CONCLUSION: In patients with complete paralysis due to neoplastic spinal cord compression, whose treatment is delayed for more than 48 hours from the onset of symptoms, surgery within one week is still beneficial. Surgery undertaken at this time may still offer neurological recovery and longer survival. The identification of the association between these factors and neurological recovery may help guide treatment for these patients. Cite this article: Bone Joint J 2019;101-B:872-879.

Spinal Epidural Venous Angioma Presenting Symtomps of Lumbar Disc Herniation: A Case Report.

Spinal epidural venous angiomas are extremely rare. We report the case of a 60-year-old man who presented with disc herniation symptoms, complaining of pain in his left leg and numbness, especially at the base of the knee. On physical examination, the Lasègue test was positive at 45 degrees on the left side and no neurological deficit was detected on the lower extremity. Contrast enhanced magnetic resonance imaging revealed a lesion in the left S1 neural foramen, which was initially evaluated as a schwannoma. However, after the lesion was totally excised, it was pathologically identified as a venous angioma. Here, the clinical presentation, management, and surgical, radiological, and pathological features are discussed.

Hodgkin Lymphoma revealed by epidural spinal cord compression.

CONTEXT: Hodgkin Lymphoma is rarely diagnosed as spinal cord compression syndrome. Caused by an epidural mass, this complication is often encountered in a late stage of the disease. We report the case of a 40-year-old man presenting with symptoms of low thoracic spinal cord compression due to an epidural tumor on the MRI. FINDINGS: Emergent surgery was undertaken on this patient, consisting in laminectomy and tumor resection. After surgery, pain relief and mild neurological improvement were noticed. The histological study revealed a Hodgkin Lymphoma and the patient was referred to chemotherapy and radiotherapy. CONCLUSION: Though chemotherapy is the gold standard treatment for Hodgkin Lymphoma, surgical spinal decompression may be required in epidural involvement of the disease. Diagnosis may be suspected in the presence of lymphadenopathy and general health decay.

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.

Prognostic Factors and a Survival Score in Patients Irradiated for Metastatic Epidural Spinal Cord Compression from Urothelial Carcinoma Cancer of the Bladder.

BACKGROUND/AIM: Prognoses of patients with metastatic epidural spinal cord compression (MESCC) from urothelial carcinoma of the bladder are generally poor. This study aimed to identify prognostic factors that can facilitate personalized care of these patients. PATIENTS AND METHODS: In 46 patients, 10 factors were evaluated for overall response (OR), post-radiotherapy (RT) ambulatory status, local control of MESCC and overall survival (OS). Independent predictors of OS were incorporated in a scoring system. RESULTS: Being ambulatory post-RT was associated with pre-RT ambulatory status (p<0.001) and better performance score (p<0.001). No factor was significantly associated with OR and local control. On multivariate analyses, lack of visceral metastases (p=0.002), being ambulatory pre-RT (p=0.001) and performance score 1-2 (p=0.004) were associated with improved OS. Based on these factors, there were three distinct prognostic groups with 0, 1-2 and 3 points and median OS times of 2, 4 and 11.5 months, respectively. CONCLUSION: Prognostic factors were identified and a new survival score was created that will help physicians aiming to personalize treatment for patients with MESCC from urothelial carcinoma of the bladder.

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.

A case of epidural glioblastoma metastasis presenting with a cervical myelopathy, torticollis, and L'hermitte's phenomenon.

Extraneural glioblastoma metastases are exceedingly rare, though previously described in the literature. Activating mutations in the BRAF kinase gene (V600E) are present in a minority of glioblastoma patients. Here, we describe a case of systemic metastases of a clonal subpopulation of BRAF V600E mutated glioblastoma in a patient previously treated with surgery, radiation, temozolomide and bevacizumab. The patient presented with a subacute cervical myelopathy during adjuvant treatment. He underwent emergent surgical decompression of an epidural spine metastasis. Analysis of the metastatic tumor demonstrated clonal expansion of a BRAF V600E subpopulation. Though rare, systemic metastasis of glioblastoma should be considered in patients presenting with subacute complaints in line with a mass lesion.