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.

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.

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.

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.

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.

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.

Impact of spinal cord compression from intradural and epidural spinal tumors on perioperative symptoms-implications for surgical decision making.

Spinal cord or cauda equina compression (SCC) is an increasing challenge in clinical oncology due to a higher prevalence of long-term cancer survivors. Our aim was to determine the clinical relevance of SCC regarding patient outcome depending on different tumor entities and their anatomical localization (extradural/intradural/intramedullary). We retrospectively analyzed 230 patients surgically treated for SCC. Preoperative status for pain and neurological impairment were correlated to the degree of compression, tumor location, and early as well as short-term follow-up outcome parameters. Interestingly, we did not observe any differences between intradural-extramedullary compared to extradural tumors. Unilaterally localized tumors were likely to present with pain (72.9 %, p < 0.01), whereas concentric growth was associated with motor deficits (41.0 %, p < 0.01, as primary symptom, 49.3 % on admission, p < 0.05). In concentric tumors, the pain pattern was diffuse (40.5 % vs. 17.5 in unilateral disease, p < 0.01), whereas unilateral tumors resulted in localized pain (61.4 % local axial or radicular, p < 0.01). Diffuse pain, patients without a sensory or motor deficit, progressive disease, cervical localization, and a higher degree of stenosis were identified as beneficial for an early improvement in pain (p < 0.05). Notably, 29 % of patients with unchanged pain and 30.8 % with unchanged neurologic function at day 7 postoperative improved during follow-up (p < 0.001). Our data demonstrate that the preoperative tumor anatomy in patients with SCC was closely related to their presenting symptoms and early clinical outcome. The detailed analysis elucidates the biology of SCC and might thereby aid in determining which patients will benefit from surgery.

Epidural spinal cavernous hemangioma and hybrid vascular subtype.

AIM: To demonstrate less common pathologies of purely epidural spinal tumors that should be considered when noted on MRI prior to surgery. To expand the differential diagnosis of purely epidural spinal tumors and comment on their surgical implications. MATERIAL AND METHODS: We report on two patients from our institution with rare pathology. We also utilized PubMed to concisely review the literature concerning purely epidural vascular lesions akin to the cavernous hemangioma. RESULTS: We describe common clinical presentations, radiographic findings, histopathologic characteristics and treatment algorithms relevant to the rare pure spinal epidural cavernous hemangioma and a newly described compound hemangioma subtype. CONCLUSION: Epidural spinal tumors are relatively common entities, though lesions isolated to the epidural space without origination in the vertebral body or as part of neurologic presentation of metastatic disease, are much less common. Less common pathologies may be missed in the initial differential diagnosis with treatment implications at surgery.

Spinal dumbbell lipoblastoma: a case-based update.

INTRODUCTION: Lipoblastomas are benign tumors that arise from the proliferation of fetal adipose tissue. They are frequently found in the extremities, in the head, and in the neck. Dumbbell conformation (which refers to epidural tumors with extraspinal extension) has been described in only seven lipoblastoma cases so far. CASE REPORT: We describe an 11-month-old patient with a large mediastinal dumbbell lipoblastoma. The mass was excised, but it recurred 1 year later when the MRI showed also multiple pleural lipoblastomatous lesions, thus establishing the diagnosis of lipoblastomatosis. The tumor has been removed with a second surgical procedure. CONCLUSION: Radical excision of lipoblastomas is the gold standard but is not always possible to achieve. Residual lesions can be managed conservatively, on the basis of the benign nature of the tumor and of its potential maturation to normal fat. Recurrence of lipoblastoma appears in 15 % of non-radical excisions but has not yet been described in the form of lipoblastomatosis. A second surgery is indicated if the tumor mass reaches large dimensions, if it increases in size, and if it threatens the functionality of vital organs such as the spinal cord.

Management of infiltrating spinal epidural angiolipoma.

Angiolipomas of the spine are rare benign tumors commonly presenting with compressive myelopathy. The present report describes a case of spinal angiolipoma with thoracic mediastinal extension in a 50-year-old woman. She presented with a long-standing history of mid-back pain with progressive lower extremities weakness. An MRI showed a heterogeneously enhancing mass located in the posterior epidural space of the thoracic spine with mediastinal extension. Histopathological examination demonstrated features consistent with spinal angiolipoma. This report emphasizes the diagnosis and therapeutic management options of infiltrating spinal angiolipomas.

Primary epidural hemangiopericytoma in the sacrum: a rare case and literature review.

Hemangiopericytoma (HPC) is an uncommon highly vascular neoplasm that originated from Zimmerman's pericytes which surrounds the endothelial tissue. Primary epidural HPC of the sacrum is extremely rare. We reported an unusual case of primary epidural malignant HPC of the sacrum that invaded vertebral bone and caused rectum compression in a 57-year-old male for the first time. The patient presented progressive low back pain and ribbon-like stool over 3 months. The surgical intervention involved sacrectomy and en bloc resection of the tumor. We described the clinical, radiological, and histological features of this tumor and reviewed the literature.

Ewing sarcoma of the thoracic epidural space in a young child.

PURPOSE: To report on the clinical course and treatment of Ewing sarcoma of the thoracic epidural space in a 5-year-old girl. METHODS: We present the case of a 5-year-old girl who experienced back pain (day 1); on day 10, the pain had exacerbated and involuntary movements in the lower limbs occurred, and an MRI performed in her local hospital revealed a tumor lesion at the upper thoracic level. RESULTS: On day 13, emergency surgery was performed for partial resection of the tumor. Pathological examination of the resected tumor by immunostaining and gene testing revealed that it was MIC2 positive and an EWS-FLI 1 chimera, respectively, and Ewing sarcoma was diagnosed. The involuntary movements resolved immediately after the surgery. Three weeks after the operation, chemotherapy and radiation therapy were commenced. After 5 months, deep tendon reflexes recovered to normal. MRI showed that the tumor has not recurred at 29 months after surgery. CONCLUSIONS: The majority of epidural patients undergo emergency surgery only after symptom exacerbation, which includes the development of neurological deficits. Thus, preoperative diagnosis of Ewing sarcoma of the epidural space is difficult and diagnosis is frequently made by a post-operative gene test. The resection area is limited to the intralesional margin area because a larger resection is difficult due to the characteristics of the affected region; thus, there is a higher possibility of recurrence and careful follow-up of the case is necessary.

Pure spinal epidural cavernous hemangioma.

BACKGROUND: Pure epidural cavernous hemangiomas without bony involvement are rare, representing 4% of all spinal epidural tumors. Most of these are case reports and are easily misdiagnosed. METHODS: Herein nine patients (male:female, 5:4, average age: 51 years) with symptomatic pure epidural spinal cavernous hemangioma between 2005 and 2011 were treated, and the clinical, radiological, and pathological records, treatment, and prognosis were discussed. RESULTS: All patients experienced a slowing progressive clinical course, except for one with intralesional hemorrhage. Clinical manifestations included back or radiating pain, sensorimotor deficits, and sphincters disturbance. Eight lesions were isointense on T1- and hyperintense on T2-weighted images with homogenously strongly enhancement and one was mixed signal with heterogeneous enhancement because of intratumoral hemorrhage. Hemilaminotomoy or laminotomy was performed and total resection was achieved. All patients experienced a gradual neurological improvement with no recurrence. CONCLUSIONS: Spinal epidural cavernous hemangioma is a benign vascular malformation that should be excluded in the diagnosis of epidural lesion. Total surgical resection is recommended and usually results in a good prognosis.

Differentiating neoplastic from nonneoplastic processes in the anterior extradural space.

PURPOSE: To compare neoplastic and nonneoplastic disease in the anterior extradural space (AES) with regard to the contour of the disease and the tethering of the central septum, as seen on axial magnetic resonance (MR) images. MATERIALS AND METHODS: In this institutional review board-approved HIPAA-compliant study, the data of patients who had AES disease determined at MR imaging and underwent diagnostic biopsy were studied. Two blinded observers reviewed the MR images and assigned the patients to one of two groups on the basis of the contour of AES disease: those with a centrally convex disease contour posteriorly and those whose disease contour was tethered in the midline to the posterior aspect of the vertebral body. Biopsy results served as the standard of reference. The two-tailed Fisher exact test, the Breslow-Day test, and κ statistics were used to compare groups. The sensitivity, specificity, and accuracy of the midline tethered contour of AES disease for the detection of neoplasm were calculated. RESULTS: The data of 32 patients (16 men, 16 women; mean age, 68 years) were studied. Seventeen patients had malignant epidural disease. Fifteen patients had nonneoplastic epidural disease: Six patients had hematomas, and nine had abscesses. A greater proportion of AES neoplasms (13 [76%] and 14 [82%] of 17 lesions for observers 1 and 2, respectively) than nonneoplastic AES lesions (four [27%] and three [20%] of 15 lesions for observers 1 and 2, respectively) demonstrated midline tethering of the central septum. For observers 1 and 2, a unilobed or bilobed appearance of AES disease had sensitivities of 76% (13 of 17 lesions) and 82% (14 of 17 lesions), respectively; specificities of 73% (11 of 15 lesions) and 80% (12 of 15 lesions), respectively; and accuracies of 75% (24 of 32 lesions) and 81% (26 of 32 lesions), respectively, for the detection of neoplasm. The mean κ value for interobserver variability was 0.62 (95% confidence interval: 0.35, 0.90). CONCLUSION: AES disease preserving the midline tethering of the central septum is more common with neoplastic disease than with infections or hematomas.

Spinal cord compression by epidural involvement over 21 vertebral levels in acute lymphoblastic leukemia.

Spinal cord compression is a rare complication of acute lymphoblastic leukemia (ALL). We report a 13-year-old boy with B-precursor ALL, presenting with restriction of breathing and back pain. Cerebrospinal fluid examination showed extremely high protein levels. Radiologic examination indicated that leukemia extended from the thoracic to sacral epidural spaces over 21 vertebral lengths in a band-shaped form, threatening to induce compressive spinal cord neuropathy. Prompt initiation of systemic chemotherapy relieved the obstruction of cerebrospinal fluid flow without local irradiation or surgical intervention. To our knowledge, this patient has shown the most extensive epidural involvement among ALL patients previously reported.

Epidural spinal tumor and periaortitis as rare complications of Wegener's granulomatosis.

This case report describes findings in a 61-year-old woman who manifested scleritis, small pulmonary nodules, otitis media, periaortitis, and progressive epidural spinal tumor, associated with elevated serum myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) levels. She was clinically diagnosed with Wegener's granulomatosis, although vasculitis was not diagnosed due to the lack of typical histological findings. We discuss the differential diagnosis in this patient, and the association of MPO-ANCA with periaortitis or epidural spinal tumor.

A persistent epidural mass in a child with B-lineage ALL.

Epidural spinal cord compression as the initial presentation of acute lymphoblastic leukemia (ALL) is a rare and serious complication. Extramedullary disease is rarely reported in patients with ALL. The most common sites are bone, followed by soft tissue, skin and lymph nodes. We describe a child with common B-lineage ALL who presented with a mass in the spinal epidural space. She was initially treated with intrathecal chemotherapy and intravenous dexamethasone with total resolution of her clinical symptoms but a persistent epidural mass. An open biopsy of the residual epidural mass was performed 7 months after diagnosis. The histological examination did not reveal any tumor infiltration, only fibrosis. We conclude that a persistent epidural mass in patients with ALL may not indicate resistant disease and may require over a year for resolution, even when response to therapy is adequate.

[Spinal epidural angiolipoma: a case report]. / Angiolipome épidural: A propos d'un cas.

Spinal epidural angiolipoma is a rare tumor revealed by a slowly progressive paraplegia. We reported a case of a 44-year-old female and point out the peculiar pattern of this lesion characterized by the prominence of the vascular component over the lipomatous component. Recognition of this entity is important because this is a benign and curable cause of paraplegia.

[Relationship of motor deficits and imaging features in metastatic epidural spinal cord compression].

OBJECTIVE: To explore the relationship of motor deficits of the lower extremities with the imaging features of malignant spinal cord compression (MESCCs). METHODS: From July 2006 through December 2008, 56 successive MESCC patients were treated at our department. All were evaluated by magnetic resonance imaging and computed tomography and were scored according to motor deficits Frankel grading on admission. Imaging assessment factors of main involved vertebrae were level of vertebral metastatic location, epidural space involvement, vertebral body involvement, lamina involvement, posterior protrusion of posterior wall, pedicle involvement, continuity of main involved vertebrae, fracture of anterior column, fracture of posterior wall, location in upper thoracic spine and/or cervicothoracic junction. RESULTS: Occurrence was the same between paralytic state of MESCCs and epidural space involvement of imaging features. Multiple regression equation showed that paralytic state had a linear regression relationship with imaging factors of lamina involvement (X1), posterior protrusion of posterior wall (X2), location in upper thoracic spine and/or cervicothoracic junction (X7) of main involved vertebrae. The optimal regression equation of paralytic state (Y) and imaging feature (X) was Y = -0.009 +0.639X, + 0.149X, +0.282X. Lamina involvement of main involved vertebrae has a greatest influence upon paralytic state of MESCC patients. CONCLUSIONS: Imaging factors of lamina involvement, posterior protrusion of posterior wall, location in upper thoracic spine and/or cervicothoracic junction of main involved vertebrae can predict the paralytic state of MESCC patients. MESCC with lamina involvement is more easily encroached on epidural space.

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.