Intradural Extramedullary Space Research Articles

MANAGEMENT OF EXTRAMEDULLARY INTRADURAL SPINAL TUMORS

Spinal tumors are relatively uncommon, accounting for approximately 5%–10% of all central nervous system tumors, with an estimated 70%–80% being located in the intradural extramedullary space. The most common intradural extramedullary tumors are schwannomas, followed by meningiomas. Other less common intradural extramedullary tumors include myxopapillary ependymomas. The clinical presentation of these tumors is often rather nonspecific, and clinicians must maintain a high index of suspicion when evaluating patients with chronic back pain. Approximately 50% of affected individuals experience nonspecific back pain, whereas radiating pain, motor deficits, and sensory loss often progress gradually. This study examines the surgical management of intradural spinal tumors treated in our department, focusing on surgical techniques Some of these benign lesions can pose a surgical challenge due to their location to spinal cord, tumor consistency (densely mineralized / calcified). Posterior approaches involving laminectomy while preserving facet joints are typically sufficient for the surgical resection of dorsal and dorsolateral tumors. In conclusion the total surgical removal of these tumors while preserving neurological function remains can pose a surgical challenge especially for those that are densely calcifed and situated anterolateral to spinal cord, but employing standard microsurgical techniques often makes these resections possible.

Spinal meningiomas.

Spinal meningiomas (SM) are lesions with a mostly favorable oncological and surgical prognosis and a low incidence of tumor recurrence. SM account for approximately 1.2-12.7% of all meningiomas and 25% of all spinal cord tumors. Typically, SM are located in the intradural extramedullary space. SM grow slowly and spread laterally into the subarachnoid space, stretching and sometimes incorporating the surrounding arachnoid but rarely the pia. Standard treatment is surgery with the primary aims of achieving complete tumor resection as well as improving and recovering neurologic function. Radiotherapy may be considered in case of tumor recurrence, for challenging surgical cases, and for patients with higher-grade lesions (World Health Organization grade 2 or 3); however, radiotherapy is mostly used as an adjuvant therapy for SM. New molecular and genetic profiling increases the understanding of SM and may uncover additional treatment options.

Spinal Cord Compression Revealing a Case of Metastasis of Burkitt Lymphoma

Burkitt lymphoma is the most frequent non-Hodgkin lymphoma in children and adolescents. The disease can involve all organs, although spinal cord involvement in the Burkitt lymphoma is exceptional and only a few cases are reported. We report a case of Burkitt lymphoma revealing by spinal cord compression in a 4-year-old patient. Magnetic resonance imaging (MRI) of the spine revealed a mass in the spinal intradural extramedullary space with severe spinal canal stenosis in T6-T9. Neuromeningeal involvement in Burkitt's lymphoma often represents a secondary location and defines an advanced stage of the disease. The child responded well to chemotherapy and is on remission.

The effect of intraoperative neurophysiological monitoring on neurological outcomes after spinal tumors operations: A single institution experience

We aim to report our study about using multimodality intraoperative neurophysiological monitoring (IONM) in intradural spinal tumor surgery. This cohort present a retrospective review of the patient who underwent intraspinal tumors surgery at Vajira Center of Excellence in Neurosurgery; between 2005 and 2020. Transcranial motor evoked potentials, somatosensory evoked potentials, and free run electromyography were used for IONM with alarm criteria. Patient record were reviews with preoperative and postoperative neurological outcome measurements; Frankel Grading, McCormick Score, American Spinal Injury Association (ASIA) Grading, Karnofsky Performance Status (KPS) Scale and The Japanese Orthopedic Association (JOA) Score at 1, 6, 12, 24 months after operation. Overall, 104 patients were operated. 77.4 % operations were used IONM. 70.2 % of tumor located at intradural extramedullary (IDEM) space and 16.7 % located at intramedullary space. There were demonstrating statistically significant (p-value < 0.050) improvement between preoperative and postoperative neurological outcome in all follow up time in IONM group. The sensitivity and specificity of alarm IONM to predict early worsening postoperative neurological outcome were 66.7 % and 88.7 %, respectively. IDEM spinal cord tumor surgery are associated with good neurological outcome (OR 0.187 [95 % CI 0.05–0.71]; p-value 0.014). Used of IONM in intradural spinal tumor surgery statistically significant improved postoperative neurological outcome and decrease postoperative neurological deficit. IONM has ability to detect postoperative neurological deficit and poor outcome with fair sensitivity and high specificity. Especially, using of IONM in IDEM have preferable postoperative neurological outcomes.

Ventral Cervical Intradural Extramedullary Neuroenteric Cyst and its Operative Procedure; A Rare Case Report with Literature Review

Background:Neuroenteric cysts are rare non-neoplastic lesions arising from a failure of dissolution of the transient neuroenteric canal between the foregut and the notochord. They are most frequently seen in the intraduralextramedullary space in the lower cervical and upper thoracic spine. Case description:A 5 yrs old boy presented to us with the complaints of neck and upper back pain and weakness of all four limbs. MRI scan shows an intraduralextramedullary cystic lesion at C6-T1 with significant compression over cord. After patient’s preparation a Posterior approach was used to remove the cysts. Post-operative course was un-eventful. Histological results were consistent with neuroenteric cysts. MRI image of 3 months follow-up shows no residual cysts and the boy has no further complaints. Conclusion:Neuroenteric cyst (NC) is a rare lesion usual location at lower cervical and upper dorsal area and should be considered among differential diagnoses. Complete excision is the treatment of choice. In most instances a dorsal surgical approach will be satisfactory. Bang. J Neurosurgery 2021; 11(1): 54-57

Spinal Cystic Schwannoma: A Rare Case Report

Spinal cystic Schwannomas are very rare tumors and have been reported in only a few case reports in literature. Our case, Mrs. Halima, a 48-year-old female was admitted in Neurosurgery department of Dhaka Medical College Hospital with the complaints of low back pain more at night occasionally radiating to right buttock and numbness and tingling of the right lower limb for 2 years. MRI of Lumbo-sacral spine revealed a large circumscribed and well delineated intradural extramedullary space occupying lesion of iso to hypointense appearance in T1WI with ring enhancement on administration of gadolinium. Per-operative observation and post-operative histopathological examination revealed schwannoma. Contrast enhanced MRI was very important for the diagnosis of this rare type of tumor and surgical planning. Surgical removal was complete and patient was recovered uneventfully.&#x0D; Bang. J Neurosurgery 2021; 10(2): 223-225

A rare case of primary high cervical intramedullary cysticercosis: Uncomplicated surgery but a preoperative diagnostic predicament

Neurocysticercosis (NCC) is the most common parasitic infection involving the central nervous system. The involvement of spine by this disease is extremely uncommon. When the spine does get affected, it generally develops as a synchronous infection with an existing cerebral cysticercosis and usually involves the intradural extramedullary space. Primary intramedullary involvement is rare. A case of primary high cervical intramedullary cysticercosis with non-progressive symptoms is described. In this case, a pre-operative diagnosis could not be ascertained. The patient underwent total surgical resection of the lesion and made excellent recovery. Post-operative evaluation did not reveal disease at other sites. This case highlights the safety and ease of surgical resection in intramedullary NCC.

Paragangliomas of the Spine.

Paragangliomas of the spine are rare tumors. Clinical presentations and courses of spinal paragangliomas are varied, and there are no standard principles of treatment to date. The purpose of this study was to explore the diagnosis, treatment and prognosis of spinal paragangliomas. The clinical data of 7 consecutive cases, with complete medical records and follow-up results that were treated in our institutions from October 2000 to October 2011, was retrospectively reviewed. There were 6 males and 1 female with a mean age of 40 years (range, 16?61). The follow-up period ranged from 40 to 98 months (mean, 72 months). Of the 6 primary spinal paragangliomas, one lesion was in the cervical intradural extramedullary space, one in the thoracic epidural space and four in the lumbar intradural extramedullary space. All tumors were totally resected and no recurrence was detected during the follow-up period. Of the metastatic case, the lesion of the spine was located in the first lumbar epidural space and vertebra. The patients underwent surgical resection two times with radiotherapy, but the tumor recurred and the patient suffered from the paraplegia of lower limbs and urine and stool incontinence during the follow-up period. No patient died. Spinal paragangliomas are rare lesions and seldom considered in the presurgical differential diagnosis due to its rarity and non-specific clinical symptoms and imaging features. Clinical follow-up was necessary to determine the outcome. Complete resection is necessary to prevent recurrence. The role of radiotherapy in the management of these lesions needs further assessment.

The Association of Leprosy and Spinal Psammomatous Meningioma: A Case Report

Leprosy or Hansen’s disease (HS) caused by Mycobacterium leprae may involve all organ systems, especially skin and nervous system, and is an contagious disease. The responsible organism is M. leprae which is an acid-fast staining bacilli and similar to M. tuberculosis . Spinal masses are rarely seen tumoral lesions. Intradural spinal masses are generally extradural located meningiomas, nerve-sheath tumors and metastasis . A 68-year-old female patient followed up by a local leprosy center was admitted for new onset lower extremity motor deficits and urinary incontinence. Cervical MRI showed the mass in size of 3 × 1.5 cm. The case was operated under general anesthesia and the lesion was located in the intradural extramedullary space with smooth border and removed totally. The pathologic result was reported as psammomatous meningioma. The purpose of this study was to present the improvement of paraparesis in the case with rarely seen lepromatous type leprosy and a spinal mass which was histologically psammomatous meningioma after emergent surgical intervention . J Med Cases. 2014;5(2):62-65 doi: http://dx.doi.org/10.14740/jmc1389w

The Association of Leprosy and Spinal Psammomatous Meningioma: A Case Report

Leprosy or Hansen’s disease (HS) caused by Mycobacterium leprae may involve all organ systems, especially skin and nervous system, and is an contagious disease. The responsible organism is M. leprae which is an acid-fast staining bacilli and similar to M. tuberculosis . Spinal masses are rarely seen tumoral lesions. Intradural spinal masses are generally extradural located meningiomas, nerve-sheath tumors and metastasis. A 68-year-old female patient followed up by a local leprosy center was admitted for new onset lower extremity motor deficits and urinary incontinence. Cervical MRI showed the mass in size of 3 × 1.5 cm. The case was operated under general anesthesia and the lesion was located in the intradural extramedullary space with smooth border and removed totally. The pathologic result was reported as psammomatous meningioma. The purpose of this study was to present the improvement of paraparesis in the case with rarely seen lepromatous type leprosy and a spinal mass which was histologically psammomatous meningioma after emergent surgical intervention. J Med Cases. 2014;5(2):62-65 doi: https://doi.org/10.14740/jmc1389w

Intradural Extramedullary Spinal Metastases of Non-neurogenic Origin

Leptomeningeal metastases from carcinoma are still poorly understood. To better define the management of unique intradural extramedullary spinal metastases (IESM) from solid cancers of non-neurogenic origin, in particular regarding leptomeningeal metastasis (LM). We conducted a retrospective, multicenter, case-control study including 11 patients with IESM matched with 11 patients with LM. Primary endpoint was overall survival; secondary endpoints were diagnostic criteria and prognostic factors. Descriptive analysis showed a clinically significant difference between IESM and LM patients regarding preexisting neurological deficit (45.5% vs 90.1%, P = .06) and malignant cells in cerebrospinal fluid (0% vs 54.5%, P = .03). The median overall survival was significantly higher for IESM patients (732 days) than for patients with LM (53 days; P < .0002). Multivariate analysis showed that preexisting neurological deficit was a negative prognostic factor for overall survival (hazard ratio: 10.2; 95% confidence interval: 1.88-102; P = .04), in contrast to functional improvement with treatment (hazard ratio: 0.01; 95% confidence interval: 0.00-0.52; P = .04). We propose the following diagnostic criteria for IESM: (1) a solid lesion located within the intradural extramedullary space, (2) the absence of other leptomeningeal lesion seen on full-spine injected magnetic resonance imaging, (3) the absence of malignant cells in cerebrospinal fluid, and (4) a histological confirmation of the metastatic nature of the lesion. The significant difference in survival between IESM and LM suggests that they are 2 distinct evolutions of the metastatic disease. Distinguishing IESM also has therapeutic consequences because patients can benefit from a focal surgical treatment with functional improvement and extended survival.

Intradural Extramedullary Ewing's Sarcoma Recurrence with Acute Clinical Presentation and Literature Review

The intradural extramedullary space is an extremely unusual site for the onset of Ewing's sarcoma. We describe a case of recurrence of intradural extramedullary Ewing's sarcoma and review the literature available on this topic.

Operative strategies in ventrally and ventrolaterally located spinal meningiomas and review of the literature

Meningiomas are usually benign tumors that arise from the meninges. They occur both intracranially and intraspinally. Spinal meningiomas account for 25–46 % of all spinal canal tumors [5, 7, 17, 21, 26]. Typically, these tumors are located in the intradural extramedullary space. Due to space occupying growth they become symptomatic over time. Initial clinical symptoms are not specific and comprise of pain, sensory deficits, motor weakness, and vegetative dysfunction, respectively [13, 14, 29]. If indicated, spinal meningiomas are usually treated by surgical resection only. Technological achievements such as preoperativemagnetic resonance imaging (MRI), intraoperative electrophysiological monitoring, and specialized microsurgical techniques have helped to improve the clinical outcome of patients suffering from spinal meningiomas in the last decades dramatically [9, 11, 17, 24, 26]. Similar to the treatment of dorsally attached tumors, the excision of ventrally attached tumors is usually performed via dorsal approaches as well, which is then hindered by the covering spinal cord [26]. Therefore, the surgical removal of such meningiomas is usually considered to be more difficult when compared to dorsally located tumors [21, 26]. In the present study, we thus investigate a large series of patients suffering from such ventrally located spinal meningiomas that were surgically treated in our department during a 20-year period.

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

Spinal intradural extraosseous Ewing's sarcoma

Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970–2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

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.

Intraspinal Clear Cell Meningioma - A Case Report -

Meningioma is one of the most common tumors of the spinal canal. Spinal meningiomas are usually found in the thoracic spine and intradural extramedullary space. Intraspinal clear cell meningiomas are a rare histological variant. Fewer than 20 intraspinal cases have been reported in the literature and only two cases have been reported in the Korean literature, but there is no report available in the Korean orthopedic literature. We report here on a case of an intraspinal clear cell meningioma that was found in the thoracic region and it was completely resected. The nonspecific MR imaging characteristics make the diagnosis of this tumor difficult. Histological examination must be used to differentiate clear cell meningiomas from other tumors. Clear cell meningioma represents an aggressive variant of meningiomas, and surgical reatment and adjuvant radiotherapy are though to be essential. Further more, long term follow-up observation will be needed for detecting recurrence of clear cell meningioma.

Dural metastases responsible for benign vertebral collapses

A 17-year-old male patient was transferred to our centre for an invasive ethmoidal carcinoma extending to both frontal lobes of the brain. Bone scintigraphy with single photon emission tomography (SPECT) showed hyperactivity at the level of the 3 to 5 and 7 to 9 vertebral bodies of the dorsal spine. Standard X-ray suggested benign bone fractures. F-fluorodeoxyglucose (FDG) positron emission tomography (PET) showed no increased uptake of the tracer in any vertebral body but there were highly hypermetabolic lesions posterior to the vertebral body at multiple levels where hyperactivity was present on the bone scan images. Magnetic resonance imaging (MRI) showed multiple metastases in the intradural extramedullary space evoking benign bone fractures, confirming the PET observations (see figure). The most probable phenomenon explaining the association of intradural metastases with benign bone fractures is vascular compression leading to vertebral infarction [1]. Indeed, vertebral infraction may accompany anterior spinal artery syndrome since the arterial supply to the dorsal part of the vertebral body derives from this artery [2]. The present observation reveals an unexpected pathophysiology for the association of bone and dural spinal lesions in advanced cancer. Classically, this association is attributed to primary bone metastasis secondarily invading the extradural space [3]. The present case unveils a possible reversed link between vertebral and dural lesions. This phenomenon is certainly far less frequent than the invasion of the extradural space by cells emanating from a vertebral metastasis. Intradural extramedullary metastasis is indeed a rare situation consequent to tumour cell spread from the blood stream and lymphatics, or from intracranial cancer sites [4], as was probably the case in our patient. Practically, the present case draws attention to the fact that “benign” vertebral collapses may be the stigmata of malignant invasion of the spinal canal in patients with cancer.

Spinal intradural extramedullary haemangioma: MRI and neurosurgical findings

Haemangiomas, have rarely been encountered in the spinal intradural extramedullary space and the MRI findings of this entity have been described only in a few cases. We present the Magnetic Resonance Imaging (MRI) and surgical findings of a rare case of intradural extramedullary cavernous angioma located at the T1-T2 level in a 65-year-old man presented progressive paraparesis and upper thoracic back pain. On MRI, a well-circumscribed intradural solid mass, 1 cm in diameter, was detected and another enhancing nodular mass was found at the nerve roots of the cauda equina. The thoracic spinal lesion was removed and the histological diagnosis confirmed cavernous haemangioma. Although very uncommon, haemangioma should be included in the differential diagnosis when a spinal intradural extramedullary lesion is discovered and some neuroradiological findings could allow a presumptive diagnosis.

Thoracic subpial intramedullary schwannoma involving a ventral nerve root: a case report and review of the literature

Background Subpial intramedullary schwannoma of the spine is a rare tumor. A few case reports have revealed that the tumor originates from around the ventral nerve exit zone, with only one case confirming involvement of the ventral root. Case Description A 72-year-old female with a 10-month history of Brown-Séquard–type monoparesis is described. On neurological examination, the left leg motor function was grade 3 or 4/5, and dysthesia with low pinprick sensation at the right side below the T8 and T9 dermatome was identified. There were no signs of multiple neurofibromatosis. Magnetic resonance imaging demonstrated a well-demarcated round mass with high enhancement and moderate peritumoral edema, but no combined syrinx. The mass (1.5 × 1 × 1.5 cm) was located at the anterior part of the spinal canal on the left of the midline of the T8 and T9 space. A left-sided unilateral approach was performed with osteoplastic laminotomy of T8 and T9 vertebrae, and radical removal of a subpial tumor was achieved. Pathological examination revealed subpial intramedullary schwannoma. The patient improved postoperatively and at discharge was able to walk without any support. Conclusion The authors emphasize that the differential diagnosis of intramedullary schwannoma should be included when peritumoral edema is moderately present to provide appropriate preoperative preparations, even if a tumor is seemingly located in the intradural extramedullary space.