Current management in the treatment of intramedullary ependymomas in children.

PURPOSE: Current management of pediatric intramedullary ependymoma is extrapolated from adult series since large studies in children are unavailable. This has led us to share our experience with this rare tumor and compare it to the literature and to review and highlight important aspects of current management and point out inconsistencies. METHODS: This is a retrospective analysis of patients with intramedullary ependymoma managed at our institution between 2004 and 2021. RESULTS: During the study period, 5 patients were treated for intramedullary ependymoma. Cases of myxopapillary ependymoma were excluded. The mean age of our cohort was 11.2 years. We identified 4 cases of grade II ependymoma and 1 case of grade III ependymoma. Gross tumor removal (GTR) was achieved in two patients (40%) of patients. One patient was treated with radiotherapy for recurrence and two patients received chemotherapy. There were no cases of recurrence among patients treated with GTR, but in all patients treated with STR. Eighty percent of patients either improved or stayed stable neurologically. During follow-up (mean 73 months), 2 patients died of disease. CONCLUSION: GTR and tumor grade remain the key prognostic factor of long-term tumor-free survival. Many questions prevail regarding outcomes, correct use of adjuvant therapy, and prognostic factors.

Approaches to Incidental Intradural Tumors of the Spine in the Pediatric Population.

BACKGROUND: Incidental intradural tumors of the spine in the pediatric population are rare lesions whose management remains unclear. Surgeons must balance the risks of iatrogenic deficits and complications after surgical resection against the risks from progressive growth of the tumor. Moreover, the natural history of an incidental finding can be difficult to predict. Here, we review the literature on incidental intradural tumors of the spine and present considerations for their management. SUMMARY: Growth of the tumor or changes in radiographic features are usually indications for resection. Asymptomatic lesions can be found in patients with genetic syndromes that predispose to tumor formation, such as neurofibromatosis type 1 and 2, schwannomatosis, and Von-Hippel-Lindau syndrome, and careful workup of a genetic cause is warranted in any patient presenting with multiple tumors and/or cutaneous features. Close follow-up is generally favored given the heavy tumor burden; however, some recommend pre-emptive resection to prevent permanent neurological deficits. Incidental intradural tumors can also occur in association with hydrocephalus, significant syringomyelia, and cord compression, and surgical treatment is usually warranted. Tumors may also be discovered as part of the workup for scoliosis, where they are not truly incidental to the scoliosis but rather are contributing to curve deformation. KEY MESSAGES: Thorough workup of patients for associated genetic syndromes or comorbidities should be undertaken in pediatric patients with incidental intradural tumors. Further research is needed into the natural history of these incidental lesions. Incidental tumors can often be managed conservatively with close follow-up, with surgical intervention warranted for expanding tumors or new-onset symptoms.

Primary spinal cord glioblastoma multiforme: a single-center experience.

INTRODUCTION: Primary spinal glioblastoma (GBM) are very rare tumors of the spinal cord, with dismal prognosis and their exact management is controversial. We attempt to formulate treatment guidelines for these extremely rare tumors based on our institutional experience and a comprehensive review of the literature. MATERIALS AND METHODS: In this retrospective study from 2008 to 2020, all the patients diagnosed with primary spinal GBM who underwent surgery at our institution were included. Clinical data were retrieved from case files, outpatient records and telephonic follow-up. Data on postoperative chemoradiation was noted in all the patients. The final diagnosis of spinal GBM was confirmed as per the histopathology reports. Patients who could not be followed up and those with prior history of cranial GBM were excluded from the study. RESULTS: Nine patients were followed up and a median survival of 11 months was noted. Chemotherapy with TMZ and radiotherapy to the whole craniospinal axis significantly improved survival in these patients. The extent of surgical resection was not shown to be significant. Intracranial metastasis was the leading cause of mortality in such patients. Three patients developed documented intracranial metastasis during the course of the disease. CONCLUSIONS: Low threshold must be kept in mind in diagnosing patients with high-grade spinal cord intramedullary tumors in view of the rapidly progressing nature of the disease. In case of positive histopathological diagnosis of spinal GBM, the whole craniospinal axis should be imaged and any cranial metastasis which was originally missed during initial workup could be given appropriate radiotherapy.

[Intraoperative ultrasound-assisted surgery of spinal tumors]. / Khirurgiya spinal'nykh opukholei s ispol'zovaniem intraoperatsionnogo ul'trazvukovogo issledovaniya.

OBJECTIVE: To improve technique of intraoperative ultrasound-assisted microsurgery of spinal tumors. MATERIAL AND METHODS: There were 68 patients with 70 spinal tumors who underwent intraoperative ultrasound-assisted resection between 2007 and 2018. Age of patients varied from 21 to 80 (mean 48.5±14.3). Intradural tumors were diagnosed in 54 (79.4%) patients (of them intramedullary in 16 (23.5%) and extramedullary in 38 (55.9%) cases). Fourteen patients (20.6%) had extradural tumors. Intraoperative ultrasound was used to determine localization, margins and structure of tumors, interrelations with neural structures, zones of dura opening and myelotomy. We also assessed quality of resection and spinal decompression. RESULTS: In surgery of spinal tumors, intraoperative ultrasound allows to localize the tumor with 95.3% sensitivity, determine the character of its growth, shape, size and internal structure. One can also differentiate the margins of neoplasm, control accuracy of approach, select the optimal zone for dura opening and myelotomy, objectively assess spinal cord and nerve roots decompression in real-time mode. Quality of intraoperative ultrasonography images is comparable to preoperative MRI, and even exceed resolution of MR scans in some cases. CONCLUSION: In our study, intraoperative ultrasound has proven to be a method complementing preoperative CT and MRI. This approach provides additional data in real-time mode to form a complete picture of surgical area, increase accuracy of manipulations and reduce surgical trauma.

Surgical treatment and neurological outcome of infiltrating intramedullary astrocytoma WHO II-IV: a multicenter retrospective case series.

INTRODUCTION: Primary malignant spinal astrocytomas present rare oncological entities with limited median survival and rapid neurological deterioration. Evidence on surgical therapy, adjuvant treatment, and neurological outcome is sparse. We aim to describe the treatment algorithm and clinical features on patients with infiltrating intramedullary astrocytomas graded WHO II-IV. METHODS: The following is a multicentered retrospective study of patients treated for spinal malignant glioma WHO II-IV in five high-volume neurosurgical departments from 2008 to 2019. Pilocytic astrocytomas were excluded. We assessed data on surgical technique, perioperative neurological status, adjuvant oncological therapy, and clinical outcome. RESULTS: 40 patients were included (diffuse astrocytoma WHO II n = 11, anaplastic astrocytoma WHO III n = 12, WHO IV n = 17). Only 40% were functionally independent before surgery, most patients presented with moderate disability (47.5%). Most patients underwent a biopsy (n = 18, 45%) or subtotal tumor resection (n = 15, 37.5%), and 49% of the patients deteriorated after surgery. Patients with WHO III and IV tumors were treated with combined radiochemotherapy. Median overall survival (OS) was 46.5 months in WHO II, 25.7 months in WHO III, and 7.4 months in WHO IV astrocytomas. Preoperative clinical status and WHO significantly influenced the OS, and the extent of resection did not. CONCLUSION: Infiltrating intramedullary astrocytomas WHO II-IV present rare entities with dismal prognosis. Due to the high incidence of surgery-related neurological impairment, the aim of the surgical approach should be limited to obtaining the histological tissue via a biopsy or, tumor debulking in cases with rapidly progressive severe preoperative deficits.

Clinical characteristics and surgical outcomes of ependymomas in the upper cervical spinal cord: a single-center experience of 155 consecutive patients.

Ependymomas occurring in the upper cervical spinal cord (above the level of the C4 segment) are rare entities with great therapeutic challenges. This study was aimed to investigate the clinicoradiological characteristics and the prognosis in a large cohort of upper cervical ependymomas from a single institution. This retrospective study enrolled 155 patients with primary ependymomas in the upper cervical spinal cord. The pre- and post-operative clinical and magnetic resonance imaging profiles were collected. The neurological outcomes and survival events were evaluated, and potential independent risk factors were analyzed. There were 82 females and 73 males, with an average age of 43.1 ± 11.3 years. Immediately post-operatively, 118 (76.1%) patients experienced neurological deterioration and 32 (20.7%) patients remained unchanged. Three months after surgery, 61 (39.4%) patients showed deteriorated neurological functions compared to the pre-operative baseline levels. After an average follow-up period of 56.0 ± 24.7 months, the neurological functions were worse than the baseline status in 37 (23.9%) patients and improved in 33 (21.3%) patients, respectively. Logistic regression analysis identified that lower age (≤ 42 years) and lower pre-operative MMS (I-II) were independent protective factors for predicting favorable neurological functions. Multivariate Cox regression analysis revealed that incomplete resection was the only independent risk factor associated with a shorter progression-free survival. Age and pre-operative functional status affect the long-term neurological outcomes, and incomplete resection was associated with a shorter survival. Our findings indicate that gross total resection should be the goal of surgical treatment of upper cervical ependymomas.

Intramedullary spinal cord metastasis from an adenoid cystic carcinoma of the external auditory canal: case report.

BACKGROUND AND IMPORTANCE: Intramedullary spinal cord metastases (ISCMs) are rare and no cases of ISCM from an adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) have been reported. CLINICAL PRESENTATION: We report a 54-year old man complaining backpain and worsening myelopathy. He had an ACC of the EAC resected years prior. A spinal MRI demonstrated a contrast-enhancing intramedullary lesion within the conus medullaris. The histopathological diagnosis of the patient was consistent with the patient's primary cancer. At 3 months follow-up, the neurological condition of the patient was unchanged. CONCLUSIONS: This is the first reported case of ISCM from a primary ACC of the EAC.

Long-term outcome and prognostic factors of intramedullary spinal hemangioblastomas.

Spinal hemangioblastomas constitute 1.6-5.8% of all spinal cord tumors. Microsurgical excision of these tumors is challenging. The purpose of this study is to analyze the neurological improvement and long-term functional outcome of spinal hemangioblastomas. This retrospective study included 15 patients who underwent surgery for intramedullary spinal hemangioblastoma at the Department of Neurosurgery of Sri Chitra Tirunal Institute for Medical Sciences and Technology from January 2001 to June 2014. Eight patients (53%) were diagnosed to have von Hippel-Lindau (vHL) disease. Eight (53%) of them were females, and seven were males (47%). Mean age was 33.8 years (16-55 years). Duration of illness ranged from 2 weeks to 4 years, and average duration was 10.5 months. Most common symptom was motor weakness followed by sensory disturbances, pain, and bladder incontinence. Six (85.7%) sporadic spinal hemangioblastomas were in McCormick grade I; whereas, 7 (87.5%) of vHL spinal hemangioblastomas were in grade II or above. In the immediate postoperative period, three patients noticed improvement in their motor weakness. Six patients (40%) experienced deterioration of preoperative neurological status in the immediate postoperative period. Three of them were sporadic tumors, and others had vHL syndrome. Favorable long-term outcome was achieved in 80% of cases. Though neurological deterioration is common after surgical resection of spinal hemangioblastomas, majority of them are reversible. Long-term functional outcome is favorable with minimal postoperative morbidities. Both sporadic- and vHL-associated tumors share common clinical and radiological features, and neurological outcome is equally good in both.

MICROSURGICAL MANAGEMENT OF LOW-GRADE SPINAL CORD ASTROCYTOMA IN ADULTS: A PERSONAL CASE SERIES REPORT AND BRIEF LITERATURE REVIEW.

Astrocytoma is the second most common intramedullary tumor of predominantly low-grade malignancy in adult patients. Adult astrocytomas have better-quality prognosis compared with astrocytomas in children. Although a standardized surgical management protocol for spinal cord glioma is currently unavailable, surgery of low-grade astrocytoma should be aimed at gross total resection to preserve neurological function and to improve the outcome. Herein, we present a personal case series of four consecutive adult spinal cord astrocytoma patients who were operated on during the last few years. Tumor resection was performed in all patients utilizing microsurgical technique and intraoperative neurophysiologic monitoring. We also provide a literature review of the treatment of intramedullary astrocytoma in adults and discuss contemporary surgical management and prognosis.

Subependymoma involving multiple spinal cord levels: A clinicopathological case series with chromosomal microarray analysis.

Subependymomas of the spinal cord are rare, do not often involve multiple levels, and very rarely recur. Here, we present a series of spinal cord subependymomas with a detailed description of the clinical, radiological and pathological features, and characterization by chromosomal microarray analysis. Briefly, the four patients included two men and two women, between the ages of 22 and 48 years. The most common presenting symptoms were neck and arm pain with upper extremity weakness. By imaging, the tumors were found to involve multiple spinal levels, including cervical/ cervico-thoracic (three patients) and thoracic (one patient), were all eccentric, and had minimal to no post-contrast enhancement. Two patients underwent gross total resection, one had a sub-total resection, and one underwent biopsy alone with a decompressive laminectomy. Follow up ranged from 6 months to 22 years. One patient (case 4) had recurrence 15 years following gross total resection and chromosomal microarray analysis revealed deletions on the long arm of chromosome 6. Our limited series suggests that spinal cord subependymomas can rarely recur, even following gross total resection, suggesting a possible role for long-term surveillance for these rare tumors.

Diagnostic errors during intramedullar processes.

OBJECTIVE: Introduction: The article describes a clinical case of a malignant tumor of the brain and spinal cord with metastasis, which remained undetected for many years and was treated as syringomyelia. Long-term exhausting examinations of the brain and spinal cord, dynamic follow-up of medical specialists, and repeated surgical interventions on the spine helped to differentiate this process and make the correct diagnosis. The aim: The objectives of the present paper are to analyze the existing classifications of syringomyelia; to examine its etiology, pathogenesis, diagnostic approaches and treatment tactics; to present a clinical case of a malignant tumor of the brain and spinal cord with metastasis, which was misdiagnosed as syringomyelia. PATIENTS AND METHODS: Materials and methods: The authors analyzed the existing classifications of syringomyelia and studied its etiology, pathogenesis, diagnostic approaches and treatment tactics using the method of content analysis, analyticalб comparative and contrastive methods. RESULTS: Clinical case: The described clinical case is a variant of the course of syringomyelia, associated with the spinal cord tumor, namely, in particular, anaplastic ependymoma. For a long time, the process remained undetected, despite the long-term examination and drainage of the syringomyelic cavity. Analyzing this case, it is highly important to pay attention to careful history collection, examination of the patient, analysis of the occurrence of certain disorders in order to conduct the neuroimaging examinations in time. CONCLUSION: Conclusions: Favorable outcome of the disease is directly related to the diagnosis at early stages, especially in young people, the clinical variant of the process, progression of the course, the degree of involvement of various parts of the nervous system and extraneural formations, the severity of the lesion. The treatment tactics and the ability to restore the functions will depend on all these factors.

Pediatric intramedullary schwannoma with syringomyelia: a case report and literature review.

BACKGROUND: Intramedullary schwannomas without neurofibromatosis are exceedingly rare. They are rarer in children with only 8 cases reported so far. The association of intramedullary schwannomas with syringomyelia is also rare. Here, we present a case of intramedullary schwannoma with syringomyelia treated surgically in an 9-year-old boy. CASE PRESENTATION: We reviewed the clinical course of a 9-year-old boy, who presented with both lower extremity weakness of 6-month duration. Neurophysical examination revealed a decreased sensation below the T10 dermatome. Magnetic resonance imaging (MRI) showed an well-demarcated intramedullary lesion located at the level of T8 vertebra with isointensity on T2WI and hypointensity on T1WI, which was homogeneous enhanced after gadolinium injection. There was associated syringomyelia extending from T7 down to the level of T10. A mild scoliotic deformity was also observed. The lesion was totally resected after an T7-T8 laminoplasty. Histopathological findings were consistent with schwannoma. Postoperative MRI did not reveal the presence of a residual tumor with syringomyelia reducted. By 2 weeks after treatment, the patient had experienced nearly complete recovery. Management with external bracing was performed on this patient for 3 months after surgery to prevent spinal deformity. However, mild spinal kyphosis occurred 5 months after surgery, and a progressive postoperative spinal kyphosis was observed during these 3 years of follow-up. Continued conservative management with observation was performed as there is no association with functional decline and impairment in health-related quality-of-life measures. CONCLUSION: Although extremely rare and uncommonly associated with syringomyelia, schwannomas need to be considered in the preoperative diagnosis of solitary intramedullary tumors in children as total resection can be achieved improving surgical outcome; Pediatric patients should be monitored closely for the development of spinal deformity following resection of intramedullary schwannoma, particularly possessing preoperative scoliotic deformity and/or tumor-associated syringomyelia.

[Surgical treatment of intramedullary-extramedullary ependymomas. Two clinical cases and a literature review]. / Khirurgicheskoe lechenie intra-ékstramedulliarnykh épendimom. Dva klinicheskikh nabliudeniia i obzor literatury.

Ependymoma is a central nervous system tumor that grows from ependymal cells lining the cerebral ventricles, central canal of the spinal cord, and filum terminale. Regardless of the histological type of ependymomas, they rarely have exophytic growth. Because of an extremely low occurrence rate of this phenomenon, we present two clinical cases of patients with classical intramedullary ependymomas (Grade II) having an extramedullary component. MATERIAL AND METHODS: The paper presents two clinical cases of patients with intramedullary-extramedullary ependymomas of the spinal cord. The surgical technique is described. After surgical treatment, the performance status of patients remained unchanged. CONCLUSION: Radical removal of complex ependymomas provides conditions for long-term disease-free survival and further neurological recovery.

Surgical treatment of sporadic and von Hippel-Lindau syndrome-associated intramedullary hemangioblastomas.

OBJECT: Intramedullary hemangioblastomas are rare lesions. They can be related to von Hippel-Lindau syndrome or they may be sporadic. This study describes surgical treatment for this infrequent tumor. METHODS: Twelve consecutive patients received surgery to remove sporadic or von Hippel-Lindau syndrome-associated intramedullary hemangioblastomas. Patients were evaluated at four time points: before treatment, on postoperative day one, on the day of discharge, and at a follow-up examination. RESULTS: The patients showed good preoperative neurological status. The cohort had a slight female predominance. All tumors spanned at least one spinal segment. In all cases, total tumor removal was achieved, and a good outcome was obtained. None of the following factors had a significant effect on outcome: age, sex, tumor size, the presence of a syrinx, or the presence of von Hippel-Lindau syndrome. CONCLUSIONS: The surgical removal of intramedullary hemangioblastomas resulted in satisfactory long-term functional outcomes. The best results were obtained before neurological symptoms occurred. Thus, we suggest that surgery should be considered for managing asymptomatic, surgically accessible, space-occupying lesions in sIH group, and isolated, space-occupying lesions in vHLS-IH group.

Discrete or diffuse intramedullary tumor? Contrast-enhanced intraoperative ultrasound in a case of intramedullary cervicothoracic hemangioblastomas mimicking a diffuse infiltrative glioma: technical note and case report.

Hemangioblastomas are benign, highly vascularized intramedullary lesions that may also extend into the intradural space. Surgery represents the standard therapy, with the goal of obtaining complete resection even at the risk of neurological morbidity. MRI is the gold standard for diagnosis and assessment of intramedullary tumors. Nevertheless, sometimes MRI may not accurately differentiate between different types of intramedullary tumors, in particular if they are associated with syringes or intra- and peritumoral cysts. This could subsequently affect surgical strategies. Intraoperative ultrasound (ioUS) has become in the last few years a very useful tool for use during neurosurgical procedures. Various ioUS modalities such as B-mode and Doppler have been applied during neurosurgical procedures. On the other hand, the use of contrast-enhanced ultrasound (CEUS) is not yet well defined and standardized in this field. We report a case of a young patient harboring a cervicothoracic intramedullary tumor, for which the preoperative neuroradiologi-cal diagnosis was in favor of a diffuse astrocytoma with nodular components whereas ioUS demonstrated 3 distinct intramedullary nodules. CEUS showed highly vascularized lesions, compatible with hemangioblastomas. These findings, particularly those obtained with CEUS, allowed better definition of the lesions for diagnosis, enhanced understanding of the physiopathological aspects, and permitted the localization of all 3 nodules, thus limiting spinal cord manipulation and allowing complete resection of the lesions, with an uneventful postoperative neurological course. To the best of our knowledge, this is the first report of the use of intraoperative CEUS in a case of intramedullary hemangioblastoma.

Disseminated spinal myxopapillary ependymoma in an adult at initial presentation: a case report and review of the literature.

Disseminated spinal myxopapillary ependymoma (MPE) is extremely rare in adults. We report a 63-year-old man with chronic low-back pain found to have multiple MPEs in the thoracic, lumbar and sacral spine. Diagnostic and management strategies of disseminated MPE are discussed with a review of pertinent literature.

Thoracic Intra and Extramedullary Capillary Hemangioma with Subacute Clinical Course: A Case Report and Literature Review.

Capillary hemangiomas are benign tumors comprising a lobulated proliferation of capillary vessels frequently located in the soft tissues of the neck and head. Spinal intradural capillary hemangiomas are rare, particularly intramedullary lesions. To our knowledge, only 31 cases of spinal intramedullary capillary hemangiomas have been reported. Here, we describe a rare case of a thoracic capillary hemangioma comprising extramedullary and intramedullary components. A 51-year-old male patient presented with bilateral lower extremity numbness and subsequent paraparesis, sensory disturbance, and bladder-bowel dysfunction with a subacute clinical course. Magnetic resonance imaging revealed a mass lesion with intramedullary and intradural extramedullary components at the Th9-10 vertebrae level and widespread spinal cord edema. Contrast-enhanced computed tomography revealed abnormal vessels on the dorsal spinal cord surface. Spinal angiography revealed a light-stained mass lesion fed by the radiculopial artery from the right Th11 intercostal artery. The tumor was resected en bloc, and the histological diagnosis was a capillary hemangioma. Postoperatively, the spinal cord edema diminished, and the patient was discharged from the convalescent rehabilitation ward. Although intramedullary capillary hemangioma is a rare spinal tumor and its preoperative diagnosis is difficult, it should be considered in the differential diagnosis of spinal intramedullary tumors.

Clinical Features and Surgical Management of Intramedullary Schwannoma of the Spinal Cord.

BACKGROUND: Intramedullary schwannoma is a relatively rare tumor with only a few literature reports. This study was aimed to report the clinical characteristics of intramedullary schwannoma and discuss imaging findings and treatment strategies. METHODS: The inclusion criterion was consecutive patients with intramedullary schwannomas who were surgically treated in our institution between 2017 and 2022. Data included clinical characteristics, radiologic features, surgical management, and prognosis. Clinical and follow-up details of all cases were collected and reviewed. RESULTS: This study included 3 male and 8 female patients. The mean age was 45 years (range 26-77 years). Cervical spine (4 cases, 36.4%), thoracic spine (4 cases, 36.4%), and lumbosacral spine (3 cases, 27.3%) involvement was found. Weakness, numbness and pain of limbs were the main symptoms at administration. Preoperative magnetic resonance imaging demonstrated lesion with spinal cord medullary invasion and well demarcated margins. The postoperative histologic examination showed benign lesions and confirmed the schwannoma. CONCLUSIONS: This article presented a series of 11 cases of intramedullary schwannoma with sharp margins and well-enhanced features. Prognosis and functional recovery were good after gross total resection.

Capillary Hemangioma of the Spinal Cord: Case Report and Systematic Review of Literature.

BACKGROUND: Capillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with intramedullary extension rare. We present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of the literature. METHODS: Medical records and imaging data were retrospectively reviewed using the health record software EPIC (Verona, Wisconsin, USA) and the radiology management software system RIS/PACS (Radiology Information System/Picture Archiving and Communication System; QREADS). The report was written in accordance with the CARE (case reports) guidelines. We also performed a systematic review of the literature on all cases of intramedullary spinal capillary hemangiomas in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. RESULTS: We report a case of a 54-year-old man who presented with progressive paraplegia and sensory deficits in the lower extremities. Spinal magnetic resonance imaging showed an intramedullary enhancing lesion centered at T11 with associated spinal cord compression. He underwent thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological examination. Histological examination showed findings consistent with a capillary hemangioma. The literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangioma. CONCLUSIONS: Purely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature. These should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients.