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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.

Objectives: To evaluate, on a long-term basis, the safety and effectiveness of percutaneous alcohol embolization (PAE) combined with percutaneous vertebroplasty (PVP) as a sole treatment for aggressive vertebral haemangiomas (AVHs) with epidural extension., Methods: From 1996 to 2015, 26 consecutive patients (15 women [58%] and 11 men; mean age 51.8 years [range: 19-75 years]) underwent PAE combined with PVP (performed at day 15) for the treatment of 27 AVHs with epidural extension. Clinical outcome was evaluated with a mean delay of 88.3 ± 53.3 months (range: 22-217 months). The primary endpoint was pain relief evaluated with a visual analogue scale (VAS)., Results: Pre-procedure mean VAS score was 7.23 ± 1.3 and significantly improved at last follow-up (m = 3.11 ± 1.9; p < 0.001). Ten patients (38.5%) remained asymptomatic. Eighty-eight percent of the patients with neurosensory disorders had complete regression of these symptoms. Two of the three patients with motor deficit did not show any improvement. No major complication was recorded., Conclusions: PAE combined with PVP is a minimally invasive safe and effective therapeutic approach for AVH with epidural involvement, even on long-term clinical outcome. This technique appears mainly effective for pain and neurosensory symptoms, but seems less effective for motor deficit relief., Key Points: • Combination of PAE with PVP is a safe technique. • PAE combined with PVP is an effective treatment for sensory symptoms. • This strategy seems less effective in patients with motor deficits.

Epidural metastases occur in 5% to 10% of cancer patients and represent a neurological emergency. Patients most commonly present with an acute onset of motor weakness, and restoration of neurological function is critically dependent on prompt diagnosis and treatment. This review discusses the clinical, epidemiological, and radiological features associated with epidural metastases and resulting spinal cord compression. Moreover, current treatment paradigms are reviewed. The timely initiation of radiation as well as surgery in select cases is critical for preserving neurological function and achieving local tumor control and pain control. Future studies investigating surgical and radiation treatment for metastatic epidural cord compression are urgently needed. Cancer 2017;123:1106-1114. © 2016 American Cancer Society., (© 2016 American Cancer Society.)

Study Design: Systematic review., Objective: To perform an evidence-based synthesis of the literature to examine the cost-effectiveness of surgery in the management of metastatic epidural spinal cord compression (MESCC)., Summary of Background Data: Between 2.5% and 10% of patients with cancer develop symptomatic MESCC, which leads to significant morbidity, and a reduction in quality and length of life. Although surgery is being increasingly used in the management of MESCC, it is unclear whether this modality is cost-effective, given the relatively limited lifespan of these patients., Methods: Numerous databases were searched to identify full economic studies based on key questions established a priori. Only economic studies that evaluated and synthesized the costs and consequences (i.e., cost-minimization, cost-benefit, cost-effectiveness, or cost-utility) were considered for inclusion. Two independent reviewers examined the full text of the articles meeting inclusion criteria to obtain the final cohort of included studies. The Quality of Health Economic Studies instrument was scored by 2 independent reviewers., Results: The search strategy yielded 38 potentially relevant citations, 2 of which met the inclusion criteria. One was a cost-utility study and the other was a cost-effectiveness study, and both used clinical data from the same randomized controlled trial. Both studies found surgery plus radiotherapy to be not only more expensive but also more effective than radiotherapy alone in the management of patients with MESCC., Conclusion: There is evidence from 2 high-quality studies that surgery plus radiotherapy is costlier but clinically more effective than radiotherapy alone for the management of MESCC. Of note, cost-effectiveness data for the role of spinal stabilization in the management of oncological spinal instability are lacking. This is a key knowledge gap that represents an opportunity for future research.

Metastatic epidural spinal cord compression is a common complication of cancer that can cause pain and potentiaLly irreversible loss of neurologic function. In most cases this syndrome is caused by compression of the thecal sac and the spinal cord by extradural metastatic mass. The most important steps in minimizing the potential neurologic sequelae are early diagnosis and rapid therapeutic intervention. MRI is generally the preferred imaging modality because of its noninvasive ability to study the entire thecal sac. Surgery and radiotherapy are the primary approaches to treat tumor compressing the spinal cord.

Neoplastic meningitis, commonly referred to as carcinomatosis meningitis, leukemic meningitis, or lymphomatous meningitis, is the third most common central nervous system metastatic complication of systemic cancer and is the most morbid of central nervous system metastases. The disease is challenging to treat for a variety of reasons, including challenges in making a diagnosis of neoplastic meningitis and lack of standardized treatment due to the paucity of clinical trials addressing treatment of neoplastic meningitis., (Copyright © 2012 Elsevier Inc. All rights reserved.)

A 26-year-old female presented with abdominal pain and distension in 2003. Clinical evaluation and imaging were suggestive of bilateral benign renal solid masses. Fine needle aspiration showed tubular cells only. Patient was kept under periodic follow up. She reported 4 years later with increase in pain and size of masses, and underwent bilateral staged nephron sparing surgery. The histopathology was reported as bilateral oncocytoma. Two years after surgery, she developed epidural spinal cord compression and liver metastasis. A decompression laminectomy and biopsy revealed conventional renal cell carcinoma (RCC). To our knowledge this is the first case report of sporadic bilateral synchronous hybrid RCC and oncocytoma in a young woman, with spinal epidural metastasis.

Purpose: To identify potential prognostic factors predicting functional outcome and survival after surgery followed by radiotherapy for metastatic spinal cord compression due to solid tumors., Methods: 531 consecutive patients with metastatic epidural spinal cord compression (MESCC) were treated at our institution. Surgery followed by radiation therapy was performed in 151 patients (30%) with various histological diagnoses. Three different surgical procedures were performed: minimal resection with or without instrumented fixation, curettage, and total tumorectomy. Within 1 month after surgery, RT was performed, delivering a total dose of 30-36 Gy (3 Gy per fraction). Ten potential prognostic factors were investigated for relationship with functional outcome and survival., Results: Clinical remission of pain was obtained in 91% of patients and 94 (62.5%) had recovery of neurological deficit. Recurrence in the same site of treatment occurred in nine (6%) patients. Median survival was 14 months (range 0-52 months); OS at 1, 2, and 3 years was 43.6, 37, and 21.5%, respectively. Survival was significantly associated with the histology of primary tumor (P < 0.001) and visceral metastases (P < 0.001) in the whole group; for histology, the prognostic factors statistically significant were other bone metastases in breast cancer, control of primary tumor, and the absence of visceral metastases in NSCLC and kind of surgery in the other., Conclusions: The key element for successful treatment of MESCC is multidisciplinary care of the patient, which includes all of those prognostic factors that have been, until now, analyzed and compared. In our set of patients treated for vertebral metastases, PS, time to development of symptoms, and the presence of visceral metastases affected functional outcome and survival.

Neoplastic metastatic epidural spinal cord compression is a common complication of cancer that causes pain and progressive neurologic impairment. The previous standard treatment for this condition involved corticosteroids and radiotherapy (RT). Direct decompressive surgery with postoperative radiotherapy (S + RT) is now increasingly being chosen by clinicians to significantly improve patients' ability to walk and reduce their need for opioid analgesics and corticosteroids. A cost-utility analysis was conducted to compare S + RT with RT alone based on the landmark randomized clinical trial by Patchell et al. (2005). It was performed from the perspective of the Ontario Ministry of Health and Long-Term Care. Ontario-based costs were adjusted to 2010 US dollars. S + RT is more costly but also more effective than corticosteroids and RT alone, with an incremental cost-effectiveness ratio of US$250 307 per quality-adjusted life year (QALY) gained. First order probabilistic sensitivity analysis revealed that the probability of S + RT being cost-effective is 18.11%. The cost-effectiveness acceptability curve showed that there is a 91.11% probability of S + RT being cost-effective over RT alone at a willingness-to-pay of US$1 683 000 per QALY. In practice, the results of our study indicate that, by adopting the S + RT strategy, there would still be a chance of 18.11% of not paying extra at a willingness-to-pay of US$50 000 per QALY. Those results are sensitive to the costs of hospice palliative care. Our results suggest that adopting a standard S + RT approach for patients with MSCC is likely to increase health care costs but would result in improved outcomes.

Background and Purpose: We present two cases of angio-proliferative tumors that were misdiagnosed and treated as typical hemangiomas with epidural expansion., Materials and Methods: Two middle-aged women presented with symptoms and radiological signs characteristic for aggressive hemangioma with epidural expansion. In the first case preoperative embolization and decompressive surgery with open transpedicular vertebroplasty was performed. Within less than a year, epidural recurrence of the tumor prompted for radical excision and corpectomy. The diagnosis after the histological studies and the further clinical evolution was metastasizing leiomyomatosis. No further recurrence occured during the next 6 years. In the second case percutaneous vertebroplasty was performed and complicated by epidural polymethyl-methacrylcate (PMMA) leakage, requiring urgent decompressive surgery. Histological study of the lesion raised the possibility of myopericytoma. This was confirmed 16 months later when complete vertebrectomy was performed due to severe epidural propagation of the recurring tumor. No further recurrence occurred in next the two years., Conclusions: Rare angio-proliferative tumors, like benign metastasizing leiomyoma and myopericytoma radiologically may resemble aggressive vertebral hemangiomas of the spine. Unlike hemangiomas, such tumors require radical removal due to their likely recurrence. As imaging studies may not be able to completely exclude such pathologies, bone biopsy and thorough histopathological studies are warranted prior to the therapeutic decision.

The occurrence of primary extraskeletal Ewing's sarcoma (EES) of the spinal epidural space has been rarely reported in the literature. The clinical, radiologic and pathologic features of a case of EES occurring in the thoracic epidural space are presented. A 37-year-old woman presented with a one-year history of back pain. Magnetic resonance imaging demonstrated an epidural mass at the T8-9 level. Laminectomy and partial resection of the tumor were performed. The differential diagnosis of a spinal epidural mass is broad. Histopathological and molecular cytogenetic examinations confirmed an EES arising from the thoracic epidural space. Despite receiving both chemotherapy and radiotherapy, the patient died of respiratory insufficiency due to medulla oblongata metastasis 22 months after the initial consultation. Awareness of this entity will allow this rare diagnosis to be considered and facilitate appropriate management. A review of the literature on spinal epidural EES is also presented.

Spinal tumors are classified according to their location in extradural, intradural-extramedullary, and intradural-intramedullary tumors. The most frequent extradural tumors are metastases. Primary spinal tumors are rare and predominantly benign. Independent of their origin, spinal tumors manifest themselves with progressive local or radicular pain and neurological deficits. A preferably early diagnosis and subsequent therapy is important to improve the prognosis. The treatment of choice for most of these tumors is the complete surgical resection. In particular with the occurrence of neurological deficits a fast surgical intervention is indicated, since the prognosis depends on duration and severity of the preoperative existing deficits. Below, clinical presentation and relevant treatment options of spinal extradural tumors are discussed.

We present our experience after analyzing retrospectively the clinicopathological characteristics, surgical approaches, immediate and long-term postoperative complications and survival outcome of pediatric spinal epidural tumors treated over a period of 10 years from 2000 to 2009 in a tertiary-care hospital. Our study included benign and malignant tumors. The majority of our patients were boys (14:8). The duration of symptoms was longer in benign lesions. Non-Hodgkin's lymphoma was the commonest malignant tumor and aneurysmal bone cyst was the commonest benign tumor. The mean duration of follow-up was 19 months (range 3-48 months). Most of the patients improved in their neurological grade after surgery; however, the degree of excision had no bearing on the length of survival for malignant lesions., (Copyright © 2011 S. Karger AG, Basel.)

Background: Myelopathy due to epidural spinal cord compression is rare in patients with malignant lymphoma and most of these patients are diagnosed with high-grade lymphoma. An epidural growth of low-grade lymphoma is even more unusual. Due to this low incidence, therapeutic experience for this entity is limited., Patients and Methods: We report the outcome of 3 consecutive patients with primary spinal epidural follicular lymphoma (FL). Due to the clinical disorders of the patients and despite the localized disease, we used an intensive multimodal therapy concept consisting of spinal decompression, systemic (immuno)chemotherapy and local irradiation. All patients improved in their medical condition; 2 achieved a complete remission, 1 of these with long-term remission., Conclusions: In contrast to the established irradiation therapy for early-stage FL, an intensive multimodal therapy concept should be initiated in patients with primary spinal epidural FL. With this approach, a fast improvement of the symptoms and long-term disease-free survival is possible., (Copyright 2010 S. Karger AG, Basel.)

A previously healthy 24-year-old male presented with a 3-month history of progressive backache and weakness in both legs. Magnetic resonance imaging of the spine showed a large soft tissue mass infiltrating paraspinal musculature of lumbosacral area, sacral laminas, last lumbar and all sacral vertebra, protruding into the spinal canal, and with propagation into pelvis. Baseline laboratory data were normal. Decompressive laminectomy and tumor removal were performed resulting in neurological improvement. Histological examination identified granulocytic sarcoma (GS). Bone marrow biopsy showed normal findings. The patient underwent adjuvant chemotherapy and radiotherapy, resulting in the elimination of residual lesion, followed by autologous transplant. Immediate diagnosis and adequate systematic treatment are essential to achieve optimal results in patients with isolated GS. The patient is alive and free of the disease 14 months from the diagnosis.

Background Context: Ewing's sarcoma is the most common childhood malignancy of bone, but it rarely occurs as a primary extraosseous epidural tumor., Purpose: To heighten awareness and treatment options of this rare (epidural) presentation of Ewing's sarcoma., Study Design: Case report., Methods: Retrospective review., Results: We present a 13-year-old boy with progressive low back and bilateral lower extremity pain and normal neurologic examination. Magnetic resonance imaging showed an extradural tumor in the dorsal epidural space from T11 to L1. He underwent a posterior laminoplasty and gross total resection of tumor; histopathological diagnosis was consistent with Ewing's sarcoma. After surgery, the patient underwent adjuvant chemotherapy and irradiation. At 10 months follow-up, the patient is neurologically intact without evidence of residual disease., Conclusions: Ewing's sarcoma of the epidural space should be considered in the differential diagnosis of epidural mass lesions. Because wide surgical margins cannot be obtained, close follow-up with a low threshold for reoperation is mandatory.

Study Design: Retrospective study of 13 patients treated by the authors., Objective: To examine the course of the disease of malignant lymphoma (ML) presenting in the epidural area of the spine., Setting: Department of Neurosurgery, Third Department of Internal Medicine, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary., Subjects and Methods: The epidural presentation in eight patients was heralded by motor signs (paraparesis and plegia), in one by a lesion of the posterior columns of the spinal cord (ataxia), and in three by pain. One patient was free of complaints and symptoms. The affected epidural area was diagnosed previously by myelography and computerized tomography (CT), and later by magnetic resonance (MR), over the course of which the location was verified as thoracic in eight patients, cervical in one, and lumbar in four. The authors recommended surgical intervention in 9 out of 13 cases, in seven cases of Hodgkin's and six cases of non-Hodgkin's lymphoma. Seven patients were treated for recognized manifestations of malignant lymphoma while six were diagnosed by intraoperative-histological examination., Results and Conclusion: The decompression operations for tumors resulted in limited improvement in seven patients (reduction in pain and return of ability to walk). Four patients were not operated on, two of which had significant improvement in their neurological symptoms. Paraparesis remained unchanged in one patient. One patient remained symptom-free. The authors emphasize the importance of interdisciplinary consultation and weighing individual priorities in the indications for operation on epidural ML.

Objective: Pediatric vertebral tumors are rare, and most of the reported series have limited numbers of cases. Diagnosis of these tumors is difficult because of the patients' age and the rarity of the lesions. We aimed to report the clinical, radiological and pathological characteristics in a small series of pediatric vertebral and spinal epidural tumors and to discuss diagnostic and treatment difficulties., Materials and Methods: Twelve consecutive pediatric cases with vertebral or spinal epidural tumors were reviewed retrospectively., Results: The mean age was 12.6 years, and male and female patients were equal in number. The most common symptom was pain. There were some neurological or local findings in all patients, and there were some positive results on plain radiographs in all cases except 2. The tumors were removed totally in 9 cases. There were histologically malignant lesions in 3 and benign lesions in 9 cases. Three patients with malignant tumors were treated by radiotherapy and 2 by chemotherapy. One patient with thoracic hemangioma was also treated by embolization after surgery. One case with cervical Dabska's tumor died due to air embolization. The other patients were followed for 48.3 months. The preoperative neurological deficits were resolved completely in all patients except the one with only biopsy performed at the last follow-up. There were no new spinal deformities on follow-up., Conclusion: Children with vertebral tumors and spinal epidural tumors usually present with pain and neurological deficits or local findings, and there are some indications on plain radiographs. Therefore, a careful physical examination and detailed evaluation of radiographs may minimize the rate of misdiagnosis and underestimation. Most of these tumors are benign, therefore, their outcome is good, and cure may be possible for many of the cases., ((c) 2008 S. Karger AG, Basel.)

Purpose: We analyzed the prevalence, clinical pattern, and prognostic impact of CNS involvement in a large cohort of children and adolescents diagnosed with non-Hodgkin's lymphoma (NHL), with special attention to differences according to NHL subtype., Patients and Methods: From October 1986 to December 2002, 2,381 patients (median age, 9.37 years; range, 0.2 to 23.8 years; female-to-male ratio, 1:2.7) from Germany, Austria, and Switzerland were registered. A total of 2,086 patients were eligible for the consecutive multicenter protocols NHL-Berlin-Frankfurt-Münster [BFM] -86, NHL-BFM-90, and NHL-BFM-95, and could be evaluated for outcome., Results: CNS involvement was diagnosed in 141 (5.9%) of 2,381 patients and was associated with an advanced stage of NHL. The percentage of CNS-positive patients was 8.8% for Burkitt's lymphoma/Burkitt's leukemia (BL/B-ALL), 5.4% for precursor B-lymphoblastic lymphoma (pB-LBL), 3.3% for anaplastic large-cell lymphoma, 3.2% for T-cell-LBL, 2.6% for diffuse large B-cell lymphoma, and 0% for primary mediastinal large B-cell NHL (P < .001). Most CNS-positive patients with pB-LBL, T-LBL, or BL/B-ALL had meningeal disease. The probability of event-free survival (pEFS; +/- SE) at 5 years was 85% +/- 1% for the 2,086 protocol patients (median follow-up, 6.5 years; range, 0.3 to 17.7 years). For the 112 CNS-positive patients, pEFS was 64% +/- 5%, compared with 86% +/- 1% for the 1,927 CNS-negative patients (P < .001). Although CNS disease had no impact on pEFS for advanced-stage T-LBL patients, CNS-positive patients with BL/B-ALL had a worse average outcome than CNS-negative patients with stage IV BL/B-ALL (60% +/- 5% v 81% +/- 3%; P < .001). In multivariate analysis, CNS disease was the strongest predictor for relapse in BL/B-ALL patients with advanced-stage disease., Conclusion: Six percent of childhood/adolescent NHL patients were CNS positive. However, the prevalence, pattern, and prognostic impact of CNS involvement differed among NHL subtypes.

Peripheral T cell lymphomas-unspecified (PTCL-U) is an uncommon malignant tumor, accounting for 5%-7% of non-Hodgkin's lymphoma. Clinical feature of a case of PTCL-U was investigated and the optimal treatment protocol was proposed. The clinical presentation of this case was progressing limbs weakness with sudden paralysis. Multiple superficial lymph nodes swelling and decreased sensation in all modalities below T10 level were found in physical examination. Bilateral leg paralysis (Grade 0/5) with high muscle tension, overactive knee reflex, bilateral Babinski sign (+) were present. Magnetic resonance imaging(MRI) showed the mass, which was around spinal cord located in T6-T8 vertebra canal, with intense enhancement after the administration of gadolinium diethylenetriamine penta-acetic acid. Abnormal signal in multiple centrum and accessories could also be found in MRI scan. Initial diagnosis was lymphoma, multiple systems involved. Laminectomy and epidural tumor resection were performed immediately. The dural sac was narrowed obviously by surrounding tumor. The result of pathological examination was PTCL-U. After operation, the level of pain sensation was descent, and touch sensation recovered. The patient received chemotherapy and his muscle strength was partly recovered in 4 months. Early diagnosis of peripheral T cell lymphomas-unspecified was difficult and easily misdiagnosed with poor prognosis. A review of literature showed central nervous system lesions occurred in advanced stage. However, the report of representation of intraspinal mass as initial symptom like the case was rare. For acute paraplegia, laminectomy and tumor resection should be performed as soon as possible. Regular radiotherapy and chemotherapy should be considered after operation. However, the value of local chemotherapy need to be further investigated.

Spinal hemangiomas can be categorized into three different groups based on location. Vertebral body (VB) hemangiomas are frequent incidental findings on magnetic resonance (MR) imaging. There is a subdivision of these with spinal epidural extension that have been reported in the literature. Spinal hemangiomas can also be epidural without VB involvement; these are extremely rare with few reported cases in the thoracic epidural spinal column. The diagnosis and imaging characteristics as well as the surgical tools used in gross-total resection of spinal epidural hemangioma are not well understood. The authors present a detailed characterization of a spinal epidural hemangioma in a 30-year-old woman who presented with complaints of gradual onset of low-back pain that worsened over 1 year. The MR imaging findings indicated a large L2-S1 epidural spinal mass causing thecal sac compression. The patient underwent an L2-S1 laminectomy, and a vascular extradural mass was noted on the posterior aspect of the dura mater. Preoperative spinal angiography as well as intraoperative angiography was performed. Total resection of the tumor was achieved using intraoperative embolization with sodium tetradecyl sulfate and microscopic dissection. The postoperative MR imaging findings and clinical outcome were excellent. The findings and use of sodium tetradecyl sulfate in gross-total resection are discussed. The authors also review treatment modalities and demonstrate the utility and effectiveness of intraoperative sodium tetradecyl sulfate in grosstotal resection of large difficult spinal epidural hemangiomas.

A rare case of extraskeletal Ewing sarcoma, arising primarily in the spinal epidural space is reported. An 18-year-old male presented with a 2-month history of right shoulder pain progressing to complete paraplegia and urinary retention over the course of 2 days. Magnetic resonance imaging demonstrated an extradural mass extending from the C6 to T1 level. Histopathologic examination confirmed the diagnosis. The literature is reviewed and radiological differential diagnosis of this rare neoplasm is briefly discussed.

Purpose: A recent randomized clinical trial has demonstrated that direct decompressive surgery plus radiotherapy was superior to radiotherapy alone for the treatment of metastatic epidural spinal cord compression. The current study compared the cost-effectiveness of the two approaches., Methods and Materials: In the original clinical trial, clinical effectiveness was measured by ambulation and survival time until death. In this study, an incremental cost-effectiveness analysis was performed from a societal perspective. Costs related to treatment and posttreatment care were estimated and extended to the lifetime of the cohort. Weibull regression was applied to extrapolate outcomes in the presence of censored clinical effectiveness data., Results: From a societal perspective, the baseline incremental cost-effectiveness ratio (ICER) was found to be $60 per additional day of ambulation (all costs in 2003 Canadian dollars). Using probabilistic sensitivity analysis, 50% of all generated ICERs were lower than $57, and 95% were lower than $242 per additional day of ambulation. This analysis had a 95% CI of -$72.74 to 309.44, meaning that this intervention ranged from a financial savings of $72.74 to a cost of $309.44 per additional day of ambulation. Using survival as the measure of effectiveness resulted in an ICER of $30,940 per life-year gained., Conclusions: We found strong evidence that treatment of metastatic epidural spinal cord compression with surgery in addition to radiotherapy is cost-effective both in terms of cost per additional day of ambulation, and cost per life-year gained.

Purpose of Review: With prolonged survival from systemic therapies in the adjuvant and salvage setting, and because these agents cannot cross the intact blood-brain barrier, central nervous system metastases are becoming a therapeutic challenge in oncology., Recent Findings: Recent therapeutic achievements include an extended use of surgery and radiosurgery. Although each of these treatment modalities has its own indications, in patients eligible for both treatments the upfront comparison of these two techniques has not been performed yet. Systemic chemotherapies and biotherapies may be effective in the management of central nervous system metastases as they may act on both neurologic and extra-central nervous system lesions. In the treatment of epidural metastases, a surgical procedure providing immediate direct circumferential decompression of the spinal cord followed by local irradiation has been demonstrated in a prospective randomized trial. The management of leptomeningeal metastases remains controversial and of limited efficacy especially in chemoresistant tumours and still relies on the combination of chemotherapy (intrathecal and intravenous) and focal radiotherapy., Summary: Aggressive treatments in patients with early diagnosis and in whom central nervous system metastases are the life-threatening location may provide a substantial increase in survival and favourably affect quality of life.

Adult Burkitt's lymphoma is an uncommon disease. Few cases of spinal involvement in adults with sporadic Burkitt's lymphoma are reported in the literature. We present a case of a middle-aged man who was found to have an epidural mass in the thoracic spine when investigated for back pain and lower extremity weakness. He underwent a laminectomy with resection of the epidural mass. Histologic examination revealed a primary Burkitt's lymphoma of the spinal cord. He was treated with aggressive chemotherapy and is now experiencing remission of his disease. We also present a review of the literature for the etiology and clinical features of other spinal lymphomas and Burkitt's lymphoma involving the spine in adults.

Spinal cord epidural metastasis (SEM) is a common complication of systemic cancer with an increasing incidence. Prostate, breast and lung cancer are the most common offenders. Metastasis usually arises in the posterior aspect of vertebral body with later invasion of epidural space. Pathophysiologically, vascular insufficiency is more important than direct spinal cord compression. The most common complaint is pain, and two thirds of patients with SEM have motor signs at initial diagnosis. Currently magnetic resonance imaging is the most sensitive diagnostic tool. The optimal management of SEM is still arguable, but recent advances in surgical management of SEM and higher complication rate of surgery following radiotherapy should persuade clinicians to consider de novo surgery where possible. Radiotherapy has an important role, particularly in treatment of radiosensitive tumors and in patients who are not candidates for surgery. Novel approaches such as stereotactic radiosurgery are promising; however, response to chemotherapy depends on inherent properties of primary tumor. Recurrent SEM is a substantial problem for which surgery or repeat radiotherapy may be options. Intramedullary metastasis is rare but should be considered in patients with systemic malignancy and asymmetrical presentation of myelopathic symptoms. The prognosis is usually poor and preferred modality of treatment is radiotherapy.

Introduction: Solid neoplasms primarily localised within parameningeal region constitute a significant diagnostic and therapeutic problem. Solid tumours developing in the primary site are soft tissue sarcomas (STS) and lymphoepithelioma., Aim of Study: Evaluation of the incidence of CNS infiltration, histological type of tumour and results of treatment., Material and Method: The study enrolled 14 patients being treated for solid tumours primarily localised within the perimeningeal region, in the Department of Paediatric Haematology and Oncology of the Medical University of Lublin between 1992 and 2004. The age of patients ranged from 2 up to 17 years (mean 8.5): 4 girls and 10 boys., Results: Soft tissue sarcoma was diagnosed in 10/14 children: rhabdomyosarcoma (RMS) embryonale in 8 patients, RMS alveolare in 1, leiomyosarcoma in 1, and lymphoepithelioma in four children. The stage of disease was III degree in all children (without CNS infiltration). Secondary infiltration of CNS was diagnosed in 2/14 children with STS., Conclusion: Secondary CNS infiltration was diagnosed in 2/14 patients. Despite of combined treatment, results of treatment are still poor due to local recurrance with CNS infiltration.

Radiotherapy has been the primary therapy for managing metastatic spinal disease; however, surgery that decompresses the spinal cord circumferentially, followed by reconstruction and immediate stabilization, has also proven effective. We provide a quantitative comparison between the "new" surgery and radiotherapy, based on articles that report on ambulatory status before and after treatment, age, sex, primary neoplasm pathology, and spinal disease distribution. Ambulation was categorized as "success" or "rescue" (proportion of patients ambulatory after treatment and proportion regaining ambulatory function, respectively). Secondary outcomes were also analyzed. We calculated cumulative success and rescue rates for our ambulatory measurements and quantified heterogeneity using a mixed-effects model. We investigated the source of the heterogeneity in both a univariate and multivariate manner with a meta-regression model. Our analysis included data from 24 surgical articles (999 patients) and 4 radiation articles (543 patients), mostly uncontrolled cohort studies (Class III). Surgical patients were 1.3 times more likely to be ambulatory after treatment and twice as likely to regain ambulatory function. Overall ambulatory success rates for surgery and radiation were 85% and 64%, respectively. Primary pathology was the principal factor determining survival. We present the first known formal meta-analysis using data from nonrandomized clinical studies. Although we attempted to control for imbalances between the surgical and radiation groups, significant heterogeneity undoubtedly still exists. Nonetheless, we believe the differences in the outcomes indicate a true difference resulting from treatment. We conclude that surgery should usually be the primary treatment with radiation given as adjuvant therapy. Neurologic status, overall health, extent of disease (spinal and extraspinal), and primary pathology all impact proper treatment selection.

Objective: Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine., Methods: A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword "hemangiopericytoma" identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine., Results: Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery., Conclusions: Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.

Purpose of Review: Metastases involving the central nervous system are notorious for the functional neurologic deficits they often cause. For many patients, the overall prognosis is determined by the systemic disease status, but a subset of patients may benefit from more intensive treatment., Recent Findings: Patients in a subgroup with brain metastases qualify for more intensive treatments, like stereotactic radiosurgery, the efficacy of which seems to be similar to that of surgery for single brain metastases. Although whole brain radiotherapy after stereotactic radiosurgery decreases the outfield recurrences, there are no indications that it improves survival. So far, studies on radiosensitizers have not resulted in clear evidence of improved outcome for good prognosis in patients with brain metastases. A variety of surgical procedures offer better palliation for a subset of patients with epidural spinal metastases than radiotherapy alone. The roles of the blood-brain barrier and of the blood-cerebrospinal fluid barrier remain a source of discussion. It may be relevant for patients with micrometastases, which can potentially be cured by systemic treatment., Summary: For many patients with central nervous system complications, adequate palliation can be achieved, but this requires rapid and thorough evaluation of patients with central nervous system symptoms. Early diagnosis and proper patient selection for further and more intensive treatment are the cornerstones of good palliative care in these patients.

The majority of patients who acquire lung cancer will have troublesome symptoms at some time during the course of their disease. Some of the symptoms are common to many types of cancers, while others are more often encountered with lung cancer than other primary sites. The most common symptoms are pain, dyspnea, and cough. This document will address the management of these symptoms, and it will also address the palliation of specific problems that are commonly seen in lung cancer: metastases to the brain, spinal cord, and bones; hemoptysis; tracheoesophageal fistula; and obstruction of the superior vena cava.

Hemangiopericytoma is an uncommon mesenchymal neoplasm that rarely affects the spinal canal. We report a case of thoracic epidural hemangiopericytoma in a 39-year-old man with symptoms of spinal cord compression studied by myelo-computed tomography (CT) and magnetic resonance imaging (MRI). Total resection was followed by complete recovery. Postoperative radiotherapy was performed. The clinical, radiological, and histological findings of epidural spinal hemangiopericytoma are reviewed in the light of relevant literature

Epidural involvement is analyzed retrospectively in 512 patients with primary treatment and follow up for Hodgkin's disease (HD) between 1970 and 1999. In one case (0.2%) epidural manifestation was the first symptom and in six cases (1.2%) it occurred later, at a disseminated, advanced stage. All seven patients were male: three had mixed cellularity and four nodular sclerosis histological subtype. The thoracic segment was involved in four cases, the lumbar in two and the cervical segment in one case. The most frequent symptoms were back pain, limb weakness, paresis/plegia, incontinence. Computer tomography, magnetic resonance imaging and myelography were used as diagnostic procedures and in planning the treatment. Functional recovery was achieved by laminectomy, loco-regional irradiation and adjuvant polychemotherapy with remission of HD for 6-100 months. Later, however, six patients died due mainly to relapse/progression of HD. We emphasize the importance of an interdisciplinary approach in the treatment of HD with this relatively rare appearance, which requires close co-operation among oncohematologists, neurologists, radiologists, neurosurgeons, radiotherapists and physiotherapists.

Non-Hodgkin's lymphoma usually involves the central nervous system by metastatic disease. Primary spinal epidural non-Hodgkin's lymphoma (PSENL) is a relatively rare cause of spinal cord compression. It mainly occurs in adults past the 4th or 5th decades. This entity is even rarer in children. The proper treatment modalities are controversial in adults with PSENL. Radiotherapy is the main strategy after surgery; the role of chemotherapy is uncertain. Therapeutic experience in childhood PSENL is extremely limited. We report a 10-year-old boy presenting with backache and bilateral lower leg weakness after minor trauma. Small non-cleaved cell non-Hodgkin's lymphoma of the epidural space was proven after subtotal tumor removal. Other investigations including computed tomography of the chest and abdomen, bone scan, gallium scan, bone marrow aspiration, and cerebrospinal fluid study were all negative for occult disease. The patient received combined therapy with irradiation and chemotherapy after surgery. Esophageal stricture resulting from radiotherapy developed during treatment and colon interposition was performed. He has remained disease free 42 months after the diagnosis with normal functional status.

Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinicopathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit--both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy.

Objective and Importance: Mantle cell lymphoma is a distinct clinicopathological type of non-Hodgkin's lymphoma that often presents at an advanced stage, with systemic spread. Spinal involvement is uncommon and generally occurs as part of advanced disease or generalized relapses. Primary spinal epidural lymphoma is a rare initial manifestation of non-Hodgkin's lymphoma, and mantle cell lymphoma with initial presentation in the spinal epidural space is extremely rare, having been previously reported in only two cases., Clinical Presentation: We report a case of a 71-year-old man who presented with increasing weakness and numbness of the legs. Magnetic resonance imaging revealed a spinal epidural mass in the lumbosacral region., Intervention: The patient underwent a partial L4 and L5-S1 laminectomy, with incomplete resection of the mass for spinal decompression and tissue diagnosis. Mantle cell lymphoma was diagnosed in the pathological examination., Conclusion: After radiotherapy, the disease recurred with a soft-tissue mass in the anterior maxillary area of the face. The patient underwent restaging and was treated with chemotherapy, with only a partial response. Mantle cell lymphoma with primary spinal epidural presentation is rare. This diagnosis can be established and other causes of spinal cord compression can be ruled out by obtaining tissue for proper histopathological examinations. Because of its aggressive behavior and poor prognosis, mantle cell lymphoma should be treated using a combined-modality approach.

We describe a case of Down's syndrome associated with progressive extradural neuroblastoma. Postmortem aspiration of the bone marrow revealed diffuse infiltration by tumor cells, in which trisomy 21 was found by fluorescence in hybridization in situ.

A 4-year-old boy presented with a rare case of an epidural tumor causing compression of the thoracic spinal cord manifesting as rapid worsening of gait disturbance. The tumor was grossly totally resected, and radiotherapy and chemotherapy were subsequently administered. The histological diagnosis was primitive neuroectodermal tumor. He has been free of recurrence for more than 6 years. Early tumor resection followed by chemotherapy and irradiation is recommended for patients with primitive neuroectodermal tumor and unstable symptoms.

Neurologic emergencies are common among cancer patients and their incidence is increasing as patients live longer as a result of improved antineoplastic therapy. This article reviews acute neurologic complications in cancer patients. Among those complications reviewed are brain metastases, epidural spinal cord compression, leptomeningeal metastases, cerebrovascular disorders, complications of antineoplastic therapy, and paraneoplastic syndromes.

We report a retrospective study regarding 6 children with primary epidural non-Hodgkin's lymphomas and analyse the factors influencing prognosis with emphasis on treatment. Four patients were females and 2 males. The neurological condition of patients was assessed pre- and postoperatively according to Shaw's classification for neurological disability. All patients were submitted to surgery, radiotherapy and chemotherapy. Histologically, the tumour proved to be a high-grade non-Hodgkin's lymphoma in all cases. At follow-up, all patients are alive after a median interval of 52 months (range 24-72 months). This study showed that the outcome of patients depends on the gravity of pre-operative deficits whereas the survival is influenced by the type of treatment.

Malignant epidural spinal cord compression (MSCC) may appear in 5-10% of adults and 3-5% of children with active malignant disease. Early diagnosis and treatment are imperative to prevent irreversible neurological damage below the level of cord compression. Unfortunately such procedures are often subjected to patient and/or doctor delay, when the emergency nature of MSCC is not understood. 3 women and 2 men, aged 17-74, are described, who illustrate both the variability of the presentation of MSCC and of its clinical course, and also problems related to imaging studies necessary to reach appropriate therapeutic decisions. These cases should increase physicians' awareness of the necessity for its early diagnosis and treatment. In addition, it is suggested that a multidisciplinary team approach be followed when MSCC is suspected (in accord with flow chart in Hebrew text).

We treated an 11-year-old girl with spinal cord compression near an epidural tumor. Bone marrow examination confirmed the diagnosis of acute lymphoblastic leukemia (ALL). To reduce the compression we treated her immediately with high-dose dexamethasone and vincristine administered intravenously along with local irradiation. Three days later, radiation was discontinued because magnetic resonance imaging showed that the spinal cord compression was reduced. Complete remission has continued without evidence of neurologic sequelae for more than 3 years since diagnosis. Rapid reduction of the blasts resulted in tumor lysis syndrome, which was treated with conventional management and additional diuresis without hemodialysis. Epidural spinal cord compression in childhood ALL can be treated effectively with systemic chemotherapy and local radiotherapy without laminectomy.

We reviewed 89 breast cancer patients with brain or epidural metastases in order to see whether a parallelism could be found between disease evolution inside and outside the central nervous system. One-fifth of the patients with brain metastases did not have any other site of relapse before neurological complication. Among the 38 patients who developed brain metastases and had a prior history of relapse outside the brain, 12 had control of constant extracranial sites while disease was progressing at these sites in 26 of them. In the epidural metastases group, all patients but 4 had progressive disease elsewhere when neurological complication appeared.

Malignant gastrinoma is a nonbeta islet cell tumor which rarely disseminates to the bone. However, in the case reported herein, diffuse metastatic bone disease with symptomatic epidural spread developed. Somatostatin and 99mTc-HDP bone scans demonstrated hot spots in the same sites, establishing that the bone lesions contained somatostatin receptor. Irradiation was effective in relieving pain.