Lumbar Spinal Epidural Capillary Hemangioma: A Case Report and Literature Review.

BACKGROUND Capillary hemangiomas are often seen on the skin of young individuals and are rarely found in the spine. These vascular lesions can arise from any spinal compartment, although they are more commonly found in the intradural extramedullary (IDEM) than the epidural location. We present a unique case of a woman with a histologically proven spinal epidural capillary hemangioma (SECH). The imaging and histopathological characteristics, as well as the treatment strategy of this vascular lesion, are highlighted along with a comprehensive review of the literature. CASE REPORT A 38-year-old woman presented with progressively worsening low back pain that radiated to both legs. Neurological examination revealed a weakness of the left leg without sensory loss. Magnetic resonance imaging (MRI) demonstrated an epidural tumor at L1-L2 level, making an obtuse angle with the cerebrospinal fluid (CSF) on sagittal T2-weighted images. The patient underwent a complete tumor resection without complications or recurrence. The histology revealed a capillary hemangioma. CONCLUSIONS SECH is exceedingly rare, with only 22 cases in the reported literature. Females are more commonly affected than males, and the thoracic spine is more commonly involved than the lumbar spine. SECH often mimics other epidural and IDEM lesions, leading to misdiagnosis. MRI is useful to differentiate SECH from lesions in the various spinal compartments; additionally, MRI is essential for preoperative planning and patient surveillance. Preoperative embolization is an option given the high vascularity of SECH. Surgery is the mainstay treatment, with a good prognosis, in most cases without recurrence.

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.

Spinal epidural capillary hemangioma: A systematic literature review and an illustrative case.

BACKGROUND: Spinal hemangiomas are rare vascular malformations resulting from proliferation of vascular endothelial cells. The cavernous form is the most common and represents 5-12% of spinal vascular malformations, while the capillary form is rare. CASE DESCRIPTION: A 56-year-old patient with no past medical history presented with progressive spinal cord compression symptoms localizing to the T10 level with MRC grade 4 proximal paraparesis. Preoperative MRI demonstrated a well-delineated, dumbbell-shaped, epidural lesion, without bony involvement, resulting in spinal cord compression at the T7 and T8 levels. The patient underwent gross total surgical resection of the lesion. At the one month follow up, the patient's strength improved to MRC grade 5, and sensation had fully returned. The histopathological diagnosis was a capillary hemangioma. Exclusively epidural capillary hemangiomas are extremely rare with only 26 cases reported in the literature. They are mainly located at the thoracic level (T4-T6). The MRI features include a well-circumscribed mass, hyperintense on T2-weighted sequence in 92% of cases, isointense on T1-weighted sequence in 88% of cases, and homogeneous contrast enhancement in 100% of cases. No tumor recurrence has been observed after gross total surgical removal. CONCLUSIONS: When evaluating progressive spinal cord compression by a purely epidural spinal lesion, the differential diagnosis should include capillary hemangioma, in addition to schwannoma, meningioma, and lymphoma. Early and complete surgical removal is the first line treatment.

Primary Paraspinal and Spinal Epidural Non-Hodgkin Lymphoma in Childhood.

Primary paraspinal/spinal epidural lymphoma (PPSEL) is rare in childhood. Here, we retrospectively evaluated patients with PPSEL treated in our department. We also reviewed the cases reported in the literature. Fifteen of 1354 non-Hodgkin lymphoma cases diagnosed over a 38-year period were PPSEPL. There were 11 male individuals and 4 female individuals with a median age of 13 years. Most common symptoms were pain and limb weakness. Physical examination revealed spinal cord compression in 80% of patients. The most common tumor location was the lumbar region. Histopathologic subtypes were lymphoblastic lymphoma in 6 and Burkitt lymphom in 5 patients. Subtotal or near-total excision of the tumor with laminectomy was performed in 6 patients. Thirteen and 9 patients received chemotherapy and radiotherapy, respectively. Neurologic recovery was observed in 70% of patients. Seven patients were alive without disease at a median of 88 months. Overall and event-free survival rates were 61.7% and 50.1%, respectively. We reviewed clinical features, treatment, and outcome of 69 PPSEL cases reported in the literature. Neurologic recovery and long-term survival was achieved in 66.7% of them. Heterogeneity in diagnostic methods and treatment have made it difficult to establish the prognostic indicators for neurologic outcome and survival. Multicenter prospective studies with more cases are necessary to determine the prognostic factors.

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.

Epidural cavernous haemangioma during pregnancy: a case report and a literature review.

Cavernous haemangiomas are benign vascular malformations that can locate in the central nervous system. The epidural spinal location remains unusual. Pregnancy is known to be a precipitating factor. The aim of this study is to review general aspects of these lesions and specific facts about their relationship to pregnancy. A 32-year-old full-term pregnant woman is managed during early labor for a progressive spinal cord compression syndrome. After delivery, exploration by a lumbar MRI found an epidural vascular dorsal mass. Surgical exploration and histopathological examination confirmed the diagnosis of epidural cavernous haemangioma. The patient achieved complete recovery after 1 month. Spinal cavernous haemangiomas are rare malformations. Specific mechanisms seems to be involved in their growth during pregnancy. Although clinical and radiological presentation are spectacular and misleading, the prognosis is generally good, and urgent surgical treatment during pregnancy is usually not indicated.

Primary paediatric epidural sarcomas: molecular exploration of three cases.

BACKGROUND: Primary paediatric epidural sarcomas are extremely rare. Overall, there remains a paucity of knowledge in paediatric epidural sarcomas owing to the infrequent number of cases. The Archer FusionPlex Sarcoma Kit (ArcherDX, Inc) is a next-generation sequencing assay that has been reported to be a useful technique to detect recurrent fusion in sarcomas. We report the molecular exploration of 3 primary paediatric epidural sarcomas-one in the cranium (mesenchymal chondrosarcoma) and 2 in the spine (mesenchymal chondrosarcoma and Ewing sarcoma respectively). CASE PRESENTATION: This is a study approved by the hospital ethics board. Clinico-pathological information from 3 consenting patients with primary epidural sarcomas was collected. These selected tumours are interrogated via Archer FusionPlex Sarcoma Kit (ArcherDX, Inc) for genomic aberrations. Results were validated with RT-PCR and Sanger sequencing. All findings are corroborated and discussed in concordance with current literature. Our findings show 2 variants of the HEY1-NCOA2 gene fusion: HEY1 (exon 4)-NCOA2 (exon 13) and HEY1 (exon 4)-NCOA2 (exon 14), in both mesenchymal chondrosarcoma patients. Next, the Ewing sarcoma tumour is found to have EWSR1 (exon 10)-FLI1 (exon 8) translocation based on NGS. This result is not detected via conventional fluorescence in situ testing. CONCLUSIONS: This is a molecularly-centered study based on 3 unique primary paediatric epidural sarcomas. Our findings to add to the growing body of literature for these exceptionally rare and malignant neoplasms. The authors advocate global collaborative efforts and in-depth studies for targeted therapy to benefit affected children.

Primary epidural hemangiopericytoma of the thoracic spine: Case report and literature review.

Hemangiopericytoma (HPC) is a rare tumor originating from pericapillary cells. Rarely found in the central nervous system, it is extremely rare in the spinal canal. Because of the low incidence of this tumor, its radiographic features and clinical manifestations have not been extensively studied and reported, therefore, it is often misdiagnosed as a schwannoma or spinal meningioma. We describe an unexpected HPC in a 35-year-old woman who was admitted to the Peking Union Medical College Hospital with a severe backache, sensory abnormalities, and muscle weakness. Magnetic resonance imaging showed an enhancing lesion at T6-7 with severe compression of the spinal cord. Gross total resection was achieved, and subsequently, a marked neurologic improvement was observed. The diagnosis of primary extradural HPC in our patient was confirmed based on postoperative histopathology and immunohistochemistry. Neither recurrence nor metastasis of the tumor was found during the 14-month follow-up, which did not include radiotherapy. To describe the demography, radiologic features, treatment, and prognosis of spinal HPC, a comprehensive literature review was performed and 105 cases of primary spinal HPC from 1958 to 2017 were collected from 39 articles. Although rare, HPC should be considered in the differential diagnosis of intraspinal lesions. Immunohistologic examination is of decisive importance in making the diagnosis. Adequate surgical resection, when feasible, is the first choice of treatment for all cases of HPC; however, the outcomes of radiotherapy and chemotherapy have yet to be determined. Individualized treatment combined with long-term follow-up for each patient is recommended.

The safety and efficacy of interstitial 125I seed implantation brachytherapy for metastatic epidural spinal cord compression.

OBJECTIVE: The objective of this study is to investigate the safety and efficacy of 125I seed interstitial implantation brachytherapy for metastatic epidural spinal cord compression (MESCC) as well as the life quality of patients. MATERIALS AND METHODS: From April 2009 to May 2015, 28 patients who met the eligibility criteria were retrospectively reviewed. The number of implanted 125I seeds ranged from 7 to 62, with appropriate activity of 0.5-0.8 mCi. The postplan showed that the matched peripheral dose (MPD) of tumors was 80-140 Gy. The duration of follow-up ranged from 1 to 32 months with a median of 18 months. Visual analog scale (VAS), Karnofsky Performance Scale (KPS), and motor performance were evaluated before and after treatment. RESULTS: Seed implantation was well tolerated by all patients. Pain was obviously alleviated in all patients. VAS score of patients was significantly decreased from 4.89 ± 1.52 before treatment to 1.61 ± 1.20 after treatment, and KPS score was significantly increased from 73.93 ± 12.27 to 86.76 ± 10.90 (P < 0.05). The local control rates of 1, 2, and 3 years were 77%, 34%, and 14%, respectively, with a median of 19 months (7-32 months). The survival rates of 1, 2, and 3 years were 81%, 54%, and 14%, respectively, with a median of 25 months. Seven (100%) nonwalking patients regained motor ability. No myelopathy or other neurologic sequelae were encountered. CONCLUSION: Interstitial 125I seed implantation brachytherapy may be a promising local therapy, which was an alternative and palliative way for treating MESCC.

Long-Term Follow-Up of Patients with Metastatic Epidural Spinal Cord Compression from Solid Tumors Submitted for Surgery Followed by Radiation Therapy.

OBJECTIVE: To evaluate the outcome of patients with epidural spinal cord compression from different solid tumors treated with a combined approach, surgery plus radiotherapy (RT), with a follow-up longer than 10 years. METHODS: Ninety-seven patients treated between 2002 and 2009 were included. Surgical treatment was performed in patients with good performance status, limited metastatic disease, life expectancy longer than 3 months, and progressive neurologic deficit and/or intractable pain. RT was performed delivering a median total dose of 30 Gy in 10 fractions. Clinical outcome was evaluated using the modified visual analog scale for pain, the Frankel scale for neurologic deficit, and magnetic resonance imaging before treatment, after treatment, and every 3 months thereafter. RESULTS: Palliative decompression was performed in 27% of patients, tumor curettage (debulking) was performed in 51%, and total vertebrectomy was performed in 22%, followed by RT in 78% of cases. Pain remission was obtained in 98% of patients, and recovery of neurologic function was obtained in 51%. The median follow-up time was 135 months (range, 96-209 months). The 5- and 10-year local control rates were 82.8% and 82.8%, respectively. The median and 5- and 10-year progression-free survival rates were 12 months, 16.9%, and 11.3%, respectively; the median and 5- and 10-year overall survival rates were 18 months, 21.3%, and 12%, respectively. On univariate and multivariate analysis, factors recorded as conditioning survival were the performance status and the presence of other metastases at the time of vertebral treatment (P < 0.01). CONCLUSIONS: Our update confirmed that surgery plus RT is a safe and feasible treatment with limited morbidity. In selected patients with favorable prognostic factors, the combined treatment may significantly impact on survival.

Bilateral Temporal Extradural Hematoma on Top of Bilateral Temporal Arachnoid Cyst: First Case Report and Extensive Literature Review.

BACKGROUND: The traumatic complication of an arachnoid cyst (AC) with intracystic and subdural hematoma has been reported in many cases. However, a unilateral extradural hematoma (EDH) as a complication of AC is extremely rare. Most arachnoid cysts are unilateral and are located in the middle cranial fossa. Bilateral temporal AC alone is an extremely rare condition, and a bilateral EDH on top of a bilateral temporal AC has never been reported in the literature, to our knowledge. CASE DESCRIPTION: We report the case of a 25-year-old man with a known bilateral AC who was involved in a motor vehicle accident and developed a bilateral temporal EDH. The brain computed tomography scan also showed fractures in the skull on both temporal areas. The patient was treated conservatively. He was discharge with Glasgow Coma Scale score of 5. CONCLUSIONS: Bilateral temporal EDH in a bilateral temporal AC has never been reported in the literature. The presence of an AC may predispose a patient to complications because of the anatomic changes in the area. It is important to educate asymptomatic patients and their families about these cysts and the importance of avoiding head injury and not being involved in contact sports or military service.

Risk Factors for Thirty-Day Morbidity and Mortality in Extradural Lumbar Spine Tumor Resection.

BACKGROUND: Epidural tumors in the lumbar spine represent a unique cohort of lesions with individual risks and challenges to resection. Knowledge of modifiable risk factors are important in minimizing postoperative complications. OBJECTIVE: To determine the risk factors for 30-day morbidity and mortality in patients undergoing extradural lumbar tumor resection. METHODS: A retrospective study of prospectively collected data using the American College of Surgeons National Quality Improvement Program database was performed. Adults who underwent laminectomy for excision of lumbar spine tumors between 2011 and 2014 were included in the study. Demographics and medical comorbidities were collected, along with morbidities and mortalities within 30 postoperative days. A multivariate binary logistic analysis of these clinical variables was performed to determine covariates of morbidity and mortality. RESULTS: The database search yielded 300 patients, of whom 118 (39.3%) were female. Overall, complications within 30 days of surgery occurred in 102 (34%) patients. Significant risk factors for morbidity included preoperative anemia (P < 0.0001), the need for preoperative blood transfusion (P = 0.034), preoperative hypoalbuminemia (P = 0.002), American Society of Anesthesiologists score 3 or 4 (P = 0.0002), and operative time >4 hours (P < 0.0001). Thirty-day mortality occurred in 15 (5%) patients and was independently associated with preoperative anemia (odds ratio 3.4, 95% confidence interval 1.8-6.5) and operative time >4 hours (odds ratio 2.6, 95% confidence interval 1.1-6.0). CONCLUSIONS: Excision of epidural lumbar spinal tumors carries a relatively high complication rate. This series reveals distinct risk factors that contribute to 30-day morbidity and mortality, which may be optimized preoperatively to improve surgical safety.

Primary Spinal Epidural CIC-DUX4 Undifferentiated Sarcoma in a Child.

Primitive round- or spindle-cell EWSR1-negative undifferentiated sarcomas harboring CIC-DUX4 gene fusion are the most common form of Ewing-like sarcomas. These tumors primarily occur in peripheral soft tissues, but examples have been described within viscera and the brain. As far as we are aware, CIC-DUX4 positive primary epidural spinal sarcoma has not been reported. Herein, we describe a T5-T6 epidural tumor in a 15-year-old girl in which many neoplastic cells had moderate and focally abundant cytoplasm, including plasmacytoid or rhabdoid cells, rather than the more common Ewing-like morphology described in the majority of such tumors. The diagnosis was confirmed by fluorescent in situ hybridization after the tumor was found to be WT-1 positive, and comprehensive genomic profiling demonstrated breakpoints in exon 20 and exon 1 of the CIC and DUX4 genes, respectively. After treatment with local radiation and systemic chemotherapy, resected recurrent tumor demonstrated more pleomorphic neoplastic cells as well as intracytoplasmic eosinophilic globules and nuclear pseudoinclusions which may reflect therapy-related changes. Unfortunately, there was further progression of tumor including the development of intracranial lesions, and the patient succumbed to her tumor 22 months after the original resection.

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.

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.

Sarcoma de Ewing extraóseo en espacio epidural / Extraosseous Ewing's sarcoma in spinal epidural space

El sarcoma extraóseo de Ewing pertenece a la familia de tumores neuroectodérmicos que derivan de la cresta neural, característico por su agresividad. Varias localizaciones se han descrito, sin embargo, la presentación epidural es rara. El pronóstico ha mejorado por nuevos esquemas oncológicos y están en estudio otras dianas terapéuticas.

Extraosseous Ewing's sarcoma belongs to the family of neuroectodermal tumors that derive from the neural crest, characteristic of its aggressiveness. Several locations have been described, however,epidural presentation is rare. Prognosis has been improved by new oncological regimens and other therapeutic targets are being studied.

Dumbbell-Shaped Epidural Cavernous Hemangioma in the Thoracic Spine Mimicking Schwannoma.

Dumbbell-shaped epidural cavernous hemangiomas (CHs) are extremely rare, and they are easily misdiagnosed as spinal schwannomas. Herein, the authors report 1 rare case of dumbbell-shaped epidural CH in the thoracic spine. To the best of our knowledge, only a few cases of dumbbell-shaped epidural CHs in thoracic spine have been reported. Furthermore, the clinical characteristics and treatments for spinal epidural CHs were investigated and reviewed.

Neck Pain in a 12-Year-Old Female: An Unusual Diagnosis.

BACKGROUND: Neck pain in the pediatric population has a broad differential diagnosis, ranging from benign to imminently life-threatening causes. Trauma and infection represent the most common etiologies of pediatric neck pain in the pediatric emergency department (PED) setting. Malignancy, though a rare cause of pediatric neck pain, is important to consider in patients with acquired torticollis or focal neurologic signs. CASE REPORT: We describe the case of a previously healthy 12-year-old female who presented to the PED with neck pain radiating down her upper extremities. The physical examination revealed diminished strength in her upper extremities compared to her lower extremities. Further evaluation revealed lymphadenopathy in the cervical and mediastinal areas and an epidural tumor in the cervical spinal column. The ultimate diagnosis was Hodgkin lymphoma presenting in an unusual manner with cervical spinal cord compression. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Neck pain is a common chief complaint among pediatric patients in the emergency setting. This case of spinal cord compression caused by malignancy illustrates the necessity of detailed spinal imaging in patients with neck pain and "red flag" signs, including but not limited to an abnormal neurologic examination.