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.

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

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

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.

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

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

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

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

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

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

Sudden Aggravated Radicular Pain Caused by Hemorrhagic Spinal Angiolipomas After Back Massage.

BACKGROUND: Spinal angiolipomas (SALs) are benign tumors that usually present a slow progressive spinal cord or radicular compression. Acute myelopathy or acute aggravated radicular syndrome are exceedingly rare. CASE DESCRIPTION: The authors report an original case with sudden aggravated radicular pain caused by hemorrhagic SALs. A 54-year-old woman presented with a 2-month history of mild back pain, and the pain was significantly aggravated after a therapeutic back massage. Neurologic examination showed pain and hyperalgesia between T4 and T7 dermatome, from back to front, just like a band. Spinal magnetic resonance imaging (MRI) showed a dorsally located epidural lesion (T4-T6) and a small intratumoral hemorrhage at the lower part of the tumor. A bilateral T4-T6 laminectomy was performed to achieve total excision of the tumors. Histologic examination showed that the tumors were composed of mature adipose tissue and vascular tissue as angiolipomas. The postoperative course was uneventful with complete neurologic recovery 4 days after the surgery. MRI at 1-year follow-up indicated no recurrence. CONCLUSIONS: SALs are unusual benign tumors that are composed of mature fatty tissue and abnormal blood vessels; sudden aggravated spinal cord or radicular compression syndrome is rare. MRI is the best choice in the diagnosis of SALs. Surgery may be performed in different ways depending on the type of SALs, and the prognosis is generally good.

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.

Spinal angiolipomas in pregnancy: Natural history and surgical treatment.

Spinal angiolipomas (SALs) are rare extradural tumors frequently located at the level of the thoracic spine and they are associated with spinal cord compromise that might result in severe myelopathy. While the first macroscopic description dates 1890, histologically these tumors where not described as angiolipomas until 1986 by Haddad et al. Occurrence in pregnancy is even more infrequent. Since their first macroscopic description, spinal angiolipomas were reported anecdotally in pregnant women. We present a case of spinal angiolipoma in pregnancy with confirmed histologic diagnosis. In the present paper, we reviewed the literature regarding spinal angiolipomas in order to characterize their clinical manifestation, natural history, radiologic and histologic appearance. We add also a further case of spinal angiolipoma in a pregnant woman. Finally, we provide suggestions for the management of such rare tumors in pregnancy.

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.

Enlarging teratoma syndrome.

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

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.

Rare Case of Dumbbell-Shaped Spinal Cavernous Hemangioma and Literature Review.

BACKGROUND: Spinal epidural cavernous hemangiomas are rare vascular malformations. Exceptionally, they present with dumbbell-shaped morphology. When they happen, it's mandatory to include their pathology in the differential diagnosis because of their similarity to schwannomas. CASE DESCRIPTION: We report the case of a 72-year-old woman with a dumbbell-shaped thoracic epidural cavernous hemangioma. A literature review of diagnostic features and current treatment options are also discussed. CONCLUSIONS: Surgery is safe and effective in both improving patient condition and preventing acute hemorrhage that can worsen the outcome, causing neurologic and potentially irreversible deficits. The favorable result we obtained in our patient suggests that surgery should be evaluated as the first option, even in patients with large epidural cavernous hemangiomas.

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.

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.

Purely Spinal Epidural Capillary Hemangiomas.

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

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.

Imaging Appearances and Pathologic Characteristics of Spinal Epidural Meningioma.

BACKGROUND AND PURPOSE: Spinal epidural meningioma is an uncommon tumor. This study aimed to analyze the imaging and pathologic characteristics of this rare tumor. MATERIALS AND METHODS: Fourteen confirmed cases of epidural meningioma were retrospectively reviewed, and imaging characteristics and pathologic findings were analyzed to identify the typical features. RESULTS: The mean age of the patients (4 men, 10 women) was 44.9 years. Twelve tumors were in the cervical spinal canal, and 2, in the thoracic spinal canal. There were 9 en plaque meningiomas, 4 dumbbell-shaped meningiomas, and 1 fusiform/ovoid meningioma. The epidural meningiomas extended over 2-5 spinal segments (mean, 3.2 spinal segments). A soft epidural mass was seen in 12/14 (86%) patients. Dural calcification was seen in 8/14 (57%) tumors. Tumor caused intervertebral foramen enlargement in 10/14 (71%) patients and adhered to the nerve roots in 11/14 (79%) patients. Intradural invasion was seen in 8/14 (57%) patients. The dural tail sign was present in 13/14 (93%) tumors on contrast-enhanced T1WI. Regarding pathologic type, 10 of 14 (71%) were psammomatous, 2 of 14 (14%) were meningothelial, 1 of 14 (7%) was angiomatous, and 1 of 14 (7%) was transitional. During follow-up (mean follow-up, 73.4 months; range, 4-192 months), 7 patients had recurrence. Recurrences were between 4 and 192 months after the operation. CONCLUSIONS: Epidural meningioma has 3 different growth patterns. Dural thickening, calcification, invasion, and epidural mass formation are characteristic features of epidural meningioma. Regular follow-up imaging is required to detect recurrence.

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

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