Thoracic exophytic ependymoma masquerading as a benign extra-axial tumor

Spinal tumors are conventionally differentiated based on location in relation to the spinal cord. Benign spinal tumors such as schwannomas and meningiomas are typically extra-axial (intradural extramedullary) lesions, whereas more aggressive primary spinal tumors such as ependymomas are typically intramedullary masses. Rarely, ependymomas can have both intramedullary and extramedullary components (typically referred to as exophytic ependymomas). We report a case of a spinal exophytic ependymoma that radiographically masqueraded as a benign intradural extramedullary lesion causing cord compression and neurologic deficit in a 47-year-old man. The diagnosis of exophytic ependymoma was made intra-operatively, with resultant gross total resection of the extramedullary portion and subtotal resection of the intramedullary portion. Histopathological examination confirmed ependymoma with World Health Organization grade II/IV. Pre-operative suspicion of an exophytic ependymoma influences operative planning and clinical management. We review the literature and discuss clinical management strategies for these interesting spinal tumors.     

Exophytic intramedullary meningioma of the cervical spinal cord

Intramedullary spinal cord neoplasms are relatively uncommon. The most common intramedullary tumors are astrocytomas and ependymomas. Meningiomas can occur as an intradural tumor; however, they are typically in the extramedullary compartment. A 42-year-old male presented with progressive sensory loss in the upper extremities and lower extremity weakness. Pre-operative imaging suggested an intramedullary cervical lesion. To treat the progressive neurological abnormality, surgical resection was planned. At surgery, it was noted that the tumor originated in the cervical spinal cord and extended into the extramedullary region. Histology confirmed the lesion to be a meningioma. This meningioma variant has not previously been described. Spinal meningiomas may occur in locations other than intradural, extramedullary locations, and should be included in the differential diagnosis of intramedullary lesions. Intramedullary meningiomas can be successfully treated with surgery.     

Surgical management of long intramedullary spinal cord tumors

Object Spinal cord tumors represent approximately 10–20% of primary central nervous system tumors. Only 20–30% of primary intradural tumors are intramedullary. The incidence of longitudinally extensive tumors involving the cervical, thoracic, and lumbar spine is very low (<1% of intramedullary lesions); hence, little literature exists on the management of this entity. Materials and methods We retrospectively reviewed all patients undergoing surgical resection of longitudinally extensive intramedullary spinal cord tumors involving the majority of the spinal cord between 1990 and 2002. Clinical, radiographic, operative, and outcome variables were retrospectively recorded and reported. Results Thirteen patients (eight male, five female) were included in the study. Mean age was 15 years (range, 3–45) at the time of the initial resection. Gross total resection was achieved in eight cases and subtotal resection in five cases. Pathology revealed astrocytoma in six cases (two pilocytic, four grade II), gangliogliomas in four cases, oligodendroglioma in two cases (one anaplastic), and lipoma in one case. One (8%) patient died from progression of anaplastic oligodendroglioma, and two (15%) underwent reoperation for recurrent tumor (ganglioglioma, grade II astrocytoma). With a mean of 3.4 years (range, 1–12) after surgery, the modified McCormick score (MMS) had worsened in only two (15%) patients, improved in three (23%) patients, and remained stable in seven (54%) patients compared to preoperative MMS. Five (38%) patients required fusion for progressive spinal deformity. Conclusion Gross total resection of holocord and longitudinally extensive intramedullary spinal cord tumors can be achieved with preservation of long-term neurological function in many cases. Serial imaging is recommended to guide subsequent resection for tumor recurrence and stabilization of progressive spinal deformity.     

Epidermoid cyst of the thoracic spine: case history

Epidermoid cysts of the spinal cord are very rare tumors. We report a 31 year-old female who presented with a 5 months history of progressive lower extremity weakness and spasticity. Magnetic resonance imaging of the thoracic spine revealed a 2 cm intradural, extramedullary mass at the T4-5 level. A T4 and T5 osteoplastic laminotomy with complete removal of the intradural mass was performed. Intraoperative and final histological examinataion revealed an epidermoid cyst. Epidermoid cysts must be a consideration for intradural, extramedullary lesions of the spinal cord. Complete surgical resection offers the patient an opportunity for good neurologic outcome.     

Exophytic ependymoma of the thoracic spine

Exophytic ependymomas of the spinal cord are very rare outside the filum or conus region. We present a patient with a thoracic spinal cord intradural extramedullary and intramedullary, World Health Organization grade II ependymoma. Gross total resection of the extramedullary component with subtotal resection of the intramedullary tumor was achieved, since there was no clear distinction between cord and tumor. The patient received postoperative external beam radiotherapy for residual tumor, and at a 2-year follow-up he is ambulatory without evidence of tumor recurrence.     

颈髓肿瘤的手术治疗

目的探讨颈髓肿瘤的手术治疗问题。方法对１５６例颈髓肿瘤（髓内１００例，髓外５６例）行手术切除，根据肿瘤部位和类型，选择手术入路和处理方法。１１例使用运动诱发电位（ＭＥＰ）技术行术中运动功能监护。采用回顾性调查的方式，对治疗方法及手术疗效进行总结。结果１５６例颈髓肿瘤手术全切率８７２％（髓内８４０％，髓外９２８％），运动功能改善率８０８％（髓内７６０％，髓外８９３％）。结论颈髓肿瘤宜积极手术治疗。对不同部位和病理类型肿瘤的切除要遵循不同的原则和方法，术中运动诱发电位监护有助于提高手术效果     

Thoracic Intramedullary Schwannoma Accompanying by Extramedullary Beads-Like Daughter Schwanommas

Thoracic intramedullary schwannomas are rare spinal cord tumors. Most of these tumors have been reported as a single lesion in the spinal cord. The authors report the first case of intramedullary schwannoma accompanying by extramedullary beads-like daughter masses of the thoracic spine. A 68-year-old male presented with walking disturbance and decreased sensation below T10 dermatome. Imaging workup revealed an intramedullary mass at T6 and T7. T6 and T7 laminectomy and mass removal were performed. Intraoperatively, extramedullary beads-like daughter masses along the nerve roots adjacent to intramedullary mass were identified. Total removal of intramedullary lesion and partial resection of extramedullary masses were done. Histological analysis confirmed the diagnosis of schwannoma. The patient could ambulate independently at postoperative 1 month without any neurological sequelae. The authors experienced a surgical case of intramedullary schwannoma accompanying by extramedullary beads-like same pathologies in the thoracic spine.     

Surgical management of intradural extramedullary tumors located anteriorly to the spinal cord

Meningiomas and nerve sheath tumors are the most common lesions found in the intradural extramedullary compartment of the spine. Some of these lesions can be located anteriorly to the spinal cord, constituting a challenge for spine surgeons. We present a surgical technique that improves the surgical exposure of lesions located anteriorly or antero-laterally to the spinal cord. A microsurgical technique of tenting of the dentate ligament with sutures and rotation of the spinal cord is described in detail and illustrated with surgical cases. This technique increases the small microsurgical operative field and allows spinal cord retraction with the use of a natural cord component, minimizing pressure on the spinal cord delicate tissue, allowing total tumor resection. In conclusion, total resection without new neurological deficit of anterior and antero-lateral tumors can be performed using an isolated posterior approach with rotation of the spinal cord using tenting of the dentate ligament with sutures.     

An intradural–extramedullary gas-forming spinal abscess in a patient with diabetes mellitus

Spinal infections are commonly reported to be located in the extradural or intramedullary spaces. Infection involving the intradural–extramedullary space are uncommon. We report a patient with uncontrolled diabetes mellitus and an infected foot ulcer who presented with a cervical cord abscess and intradural gas. Early diagnosis and aggressive treatment are necessary for a favourable outcome in gas-forming intradural spinal abscesses. To our knowledge, a gas-forming intradural spinal abscess has not been reported previously and we discuss the relevant literature.     

Neoplastic myelopathy

Neoplastic myelopathy may be due to external compression or to direct intraparenchymal involvement of the spinal cord. In this review, the authors discuss the most common cause for compressive neoplastic myelopathy, metastatic disease. They also review other compressive lesions and discuss primary intramedullary spinal tumors. In the acute setting, compressive metastatic disease should be treated with high-dose steroids when clinically necessary; surgery should be considered for selected patients, followed by radiation therapy. For most primary intramedullary spinal tumors, surgical resection remains the standard initial therapy. Patients with incomplete resection of infiltrative tumors, high-grade pathology, or recurrent tumors may benefit from radiation, but most spinal tumors are relatively insensitive to traditional chemotherapy. Neoplastic myelopathy from either compressive or intraparenchymal causes remains a diagnostic and therapeutic challenge. In complex cases, referral to a specialty center with access to neurosurgeons, neuroradiologists, neuropathologists, and neurooncologists is recommended.     

高颈段椎管内肿瘤的显微外科治疗

目的分析高位颈段椎管内肿瘤病例特点，探讨其手术方法和疗效。方法22例高位颈段椎管内肿瘤中，位于髓内者5例．位于髓外硬脊膜下者17例，其中有11例呈哑铃形生长。全部患者均行显微神经外科手术治疗，对于颈髓受压较严重者手术后采用大剂量甲基强的松龙治疗。结果本组5例髓内肿瘤中，3例室管膜瘤全切除，1例星形细胞瘤行部分切除，另1例星形细胞瘤行椎板内减压后活检。17例髓外硬脊膜下肿瘤中，有15例均在显微镜下全切除，有2例行大部切除；其中呈哑铃形生长的11例肿瘤中．有9例获得显微镜下全切除。结论术前MRI对高颈段椎管肿瘤有较好的诊断价值，而显微神经外科手术则有助于此类肿瘤的全切除。     

Isolated spinal cord lipoma

A case of isolated intradural spinal cord lipoma is presented. Most isolated spinal cord lipomas are intradural extramedullary, and the most common location is subpial. True intramedullary lipomas are very rare. Patient history is usually of months to years of local back pain, with recent escalation of pain and development of neurological symptoms. MRI examination shows a well circumscribed lesion of high signal on both T1 and T2 weighted images, and suppression on fat saturation sequence. Subtotal resection is the surgical aim. This improves pain but neurological symptoms rarely improve, usually are unchanged, and occasionally are worse.     

Tanycytic ependymoma in association with neurofibromatosis type 2.

A rare case of tanycytic ependymoma associated with neurofibromatosis type 2 (NF2) is presented for the first time, with emphasis on its clinical course and histopathological features. A 30-year-old man had developed gait disturbance in his childhood, and harbored multiple tumors in spinal nerve roots, in the intradural extramedullary and intramedullary spinal cord. The spinal root tumor and intradural extramedullary tumor were histologically diagnosed as schwannoma and meningioma, respectively. Magnetic resonance imaging showed two intramedullary cystic lesions, one in the cervical and the other in the thoracic spine. Because his sensorimotor dysfunction in the lower extremities continued to worsen gradually, three of the multiple nodular tumors in the thoracic cystic lesion were removed. All three tumors were composed of eosinophilic piloid cells with modest nuclear pleomorphism. No Rosenthal fibers were found. A concentration of slender eosinophilic cellular processes surrounding the vascular wall was seen. Periodic acid Schiff and Masson trichrome-positive balloons were seen in the extracellular space. Detection of ependymal rosettes, although only few in number, led the diagnosis as a tanycytic ependymoma. Recognition of this ependymoma variant should be emphasized to avoid confusion with pilocytic astrocytoma or intramedullary schwannoma.     

Surgical technique and outcome in cervical and thoracic myelomeningocoele surgery.

Cervical and thoracic myelomeningocoeles differ from common lumbosacral myelomeningocoeles in many respects. We review the surgical technique and outcome achieved for a series of six infants who underwent surgery for cervical or thoracic myelomeningocoele. Five patients, who had intradural exploration and microsurgical untethering of the spinal cord, were neurologically stable on follow-up. The other patient, who had a simple subcutaneous resection of the sac without release of the intradural tethering bands, was re-operated on 16 months later, with progressive neurological symptoms due to cord tethering. Following re-exploration and microsurgical untethering of the spinal cord, the neurological deficits significantly improved. We suggest that the surgical technique in these lesions should include careful intradural exploration and microsurgical release of the spinal cord by meticulous resection of all tethering bands. This enables postoperative neurological improvement and possible prevention of future neurological deficits due to cord tethering.     

Spinal subdural epidermoids - a separate entity: report of 3 cases

Intradural extramedullary epidermoid cysts are rare tumors especially those not associated with spinal dysraphism. We report 3 cases of spinal intradural extramedullary epidermoid cysts. In all the cases, the lesion was situated at dorsal level. The clinical features, MRI characteristics and surgical treatment of such rare intradural extramedullary benign tumors are discussed and relevant literature is reviewed.     

Minimally Invasive Removal of an Intradural Cervical Tumor : Assessment of a Combined Split-Spinous Laminectomy and Quadrant Tube Retractor System Technique

Conventional laminectomy is the most popular technique for the complete removal of intradural spinal tumors. In particular, the central portion intramedullary tumor and large intradural extramedullary tumor often require a total laminectomy for the midline myelotomy, sufficient decompression, and adequate visualization. However, this technique has the disadvantages of a wide incision, extensive periosteal muscle dissection, and bony structural injury. Recently, split-spinous laminectomy and tubular retractor systems were found to decrease postoperative muscle injuries, skin incision size and discomfort. The combined technique of split-spinous laminectomy, using a quadrant tube retractor system allows for an excellent exposure of the tumor with minimal trauma of the surrounding tissue. We propose that this technique offers possible advantages over the traditional open tumor removal of the intradural spinal cord tumors, which covers one or two cervical levels and requires a total laminectomy.     

颈髓肿瘤的显微外科治疗

目的 探讨颈髓肿瘤的显微外科治疗方法. 方法 根据肿瘤的不同部位和病理类型,对36例肿瘤(髓内12例,髓外24例)选择不同的手术人路和方法.结果 髓内肿瘤12例中10例获得全切,7例术后神经功能明显改善或稳定.髓外肿瘤24例中22例获得全切,23例术后神经功能明显改善或稳定.结论 颈髓肿瘤应采用显微外科技术治疗,能有效的保护脊髓组织及神经,减少神经功能障碍的进一步加重,提高手术全切率,改善预后.手术入路的选择应根据肿瘤的部位、大小、发展方向和病理类型选择不同的手术入路.     

Giant Ventral Midline Schwannoma of Cervical Spine : Agonies and Nuances

Pure ventral midline giant schwannoma is an extremely rare entity. Spinal intradural extramedullary schwannomas commonly occur posterolateral or anterolateral to the spinal cord. A case of a pure midline ventrally situated giant pan cervical extramedullary schwannoma in an 18-year-old male patient with compressive myelopathy and sphincter involvement is presented. Spinal MR imaging showed a midline ventrally situated extramedullary tumor with severe spinal cord compression extending from clivus to C7 vertebra. It was resected through a posterolateral approach. Histology was consistent with a schwannoma. Post operative MR imaging showed no evidence of the tumor. The radiological features, pathogenesis and surgical strategies in management of these difficult tumors are discussed and the relevant literature is briefly reviewed.     

Surgical treatment and outcomes of intramedullary tumors by minimally invasive approach

Intramedullary tumors are uncommon neoplasms which, without treatment, can cause neurologic morbidity or mortality. The goal of the treatment is complete surgical resection with a minimally invasive approach while preserving neurological status and also spinal stability. Out of 1972 patients with tumors of the spinal canal treated between 1994 and 2017, 168 intramedullary tumors of 417 intradural tumors have been presented. All patients had undergone one surgical resection. The mean age is 43 ± 12 years (range 11–67 years). Tumors were subdivided into 4 groups: cervically located-tumors (n = 43), cervicothoracic-region-tumors (n = 32), thoracic-region-tumors (n = 57), and lumbosacral-region-tumors (n = 36). The mean follow-up time was 37 ± 29 months. Gross-total resection rate was higher in cervical located intramedullary tumors compared to the thoracic intramedullary tumors. Cervical intramedullary tumors showed better postoperative functional outcome than the thoracic intramedullary lesions. In intramedullary tumors, extending more than 3 spinal segments, postoperative worsening was significantly increased. A minimally invasive approach (the bilateral decompression via unilateral hemilaminectomy) was used to remove the tumor while preserving spinal stability. Perioperative permanent morbidity was very low. Intramedullary tumors should be surgically treated as soon as neurological symptoms appear. Patients with thoracic intramedullary tumors and tumor extension of more than three segments were at a higher risk for permanent morbidity. The minimally invasive approach allowed complete removal of the intramedullary tumors, and adequate preservation of vertebral stability while providing a good postoperative course.     

Cervical intradural lipoma with associated hemivertebra formation at C6 level: a case report

INTRODUCTION AND CASE DESCRIPTION: Intramedullary lipomas are rare tumours of the spinal cord and account for about 1% of all spinal neoplasms. These lesions can occur anywhere along the length of spinal cord, but are frequently localized to the lower thoracic and lumbosacral levels. The authors present a 18-year-old female with intractable shoulder and neck pain and progressive weakness in the upper extremities, harbouring a cervical intradural lipoma with intramedullary extension, along with concomitant scoliosis. CONCLUSION: Despite its benign nature, surgical treatment of these lesions in symptomatic patients generally provides satisfactory relief of symptoms. Radical removal of spinal intradural lipomas is not recommended since attempts at complete excision carry an unacceptable risk of postoperative morbidity and sufficient decompression with or without duraplasty generally provides a successful clinical outcome.