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.

Diagnosis and treatment of epidural metastases.

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.

Long-term outcome of percutaneous alcohol embolization combined with percutaneous vertebroplasty in aggressive vertebral hemangiomas with epidural extension.

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.

Cost-effectiveness of surgery in the management of metastatic epidural spinal cord compression: a systematic review.

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.

[Paradigm shift in the management of metastatic epidural spinal cord compression: the importance of preserving ambulation].

In 2005, a Landmark study showed that direct decompressive surgery, followed by postoperative external beam radiotherapy (EBRT) is superior to EBRT alone in patients with metastatic epidural spinal cord compression (MESCC). Patients undergoing both surgery and EBRT had similar median survival but experienced longer ambulation than with EBRT alone. Additional studies have shown improvements in quality-of-life, higher cost-effectiveness, improved pain control, and higher functional status with surgery plus EBRT. Improved neurological outcome also improved the patients' ability to undergo postoperative adjuvant therapy. According to our experience, even patients over 65 or patients with aggressive primary tumors and additional metastases have benefited from surgical intervention, living longer than expected with preservation of ambulation and sphincter control until death or shortly before. Preserving ambulation is critical. With current surgical devices and techniques, patients with MESCC who present with a single area of cord compression, back pain, neurological deficit, or progressive deformity, may benefit from surgery prior to adjuvant radiation-based treatment or chemotherapy.

[Metastatic spinal cord compression: diagnosis and treatment].

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 and metastatic epidural spinal cord compression.

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.

The combined use of surgery and radiotherapy to treat patients with epidural cord compression due to metastatic disease: a cost-utility analysis.

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.

Assessment of prognostic factors in patients with metastatic epidural spinal cord compression (MESCC) from solid tumor after surgery plus radiotherapy: a single institution experience.

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.

Rare angioproliferative tumors mimicking aggressive spinal hemangioma with epidural expansion.

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.

Bilateral hybrid oncocytoma and renal cell carcinoma.

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.

[Spinal tumors - part 1: extradural tumors. Epidemiology, clinical aspects and therapy]. / Spinale Tumoren - Teil 1: Extradurale Tumoren. Epidemiologie, klinische Aspekte und Therapie.

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.

Pediatric epidural tumors of the spine--experience of a decade from the Kashmir Valley.

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.

Extraskeletal Ewing's sarcoma of the thoracic epidural space: case report and review of the literature.

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.

Multimodal therapy for localized spinal epidural follicular lymphoma.

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.

Primary thoracolumbar Ewing's sarcoma presenting as isolated epidural mass.

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.

Spinal epidural granulocytic sarcoma in non-leukemic patient.

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.

Pediatric vertebral and spinal epidural tumors: a retrospective review of twelve cases.

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.