Primary spinal primitive neuroectodermal tumor: A single center series with literature review

Context: Primary spinal primitive neuroectodermal tumor (PNET) of the central nervous system has a low incidence. The intraspinal case is very rare. Around 30 cases have been reported so far. We summarized the cases of primary spinal PNET available in the database of our institute, either intramedullary or extramedullary cases. Then we did literature review of the same disease.Findings: There were eight cases of primary spinal PNET available in our database, with one intramedullary case and seven extramedullary cases. Surgical resection was performed. The histology diagnosis was PNET. Peri-operative image examinations of the whole central nervous system (CNS) were performed to exclude tumors other than spinal cord origin. Then during literature review, 33 reports of the disease were included. The pre-operative diagnosis rate was low. The disease had a high recurrence rate and poor prognosis given available treatment.Conclusion: Primary spinal primitive neuroectodermal tumor is of high malignancy. Little is known due to its quite low incidence. The prognosis is poor due to lacking of effective treatment strategy. Present treatment strategy is referred to other common CNS malignancies like glioma. Further investigation of the disease is necessary.     

Intramedullary epidermoid associated with an intramedullary spinal abscess secondary to a dermal sinus.

Intramedullary spinal abscesses are rare and potentially devastating lesions. A heightened clinical awareness of patients at risk is essential for an early diagnosis and intervention to improve the prognosis of these patients. The first case of spinal abscess was described in 1830, and only 63 cases have been reported subsequently. Intraspinal epidermoids also are unusual lesions. These tumors were reported first in 1829 and represent less than 1% of all intraspinal tumors. We present a case of a 2-year-old girl who had rapidly progressive paraplegia with urinary and bowel retention and was found to have an intramedullary epidermoid and an intramedullary abscess as a result of a dermal sinus. The association of an intramedullary abscess and epidermoid tumor, two rare lesions, has not been reported previously. We also review the literature related to both intramedullary epidermoids and intramedullary abscesses of the spinal cord. Advances in sectional imaging as described should help lead to an early and safe diagnosis of these rare but often debilitating lesions. This case illustrates the importance of a complete neurological assessment of all patients with a congenital dermal sinus because of the potential for intradural extension and the frequent association with other dysraphic abnormalities. Prophylactic surgery is indicated in many cases to prevent dangerous and recurrent infections of the central nervous system.     

Intramedullary spinal cord metastasis associated with hemorrhage: a case report

A rare case of intramedullary spinal cord metastasis associated with hemorrhage was reported. A 74-year-old man had a subacute onset of paraparesis. He became almost paraplegic within a few days. MRI revealed an intramedullary spinal lesion in the epiconus at the Th 11 and Th 12 level, but spinal angiography did not show any abnormality. Since repeated MRI showed enlargement of the lesion, surgery was performed under the diagnosis of an intramedullary spinal cord tumor. Under general anesthesia, a midline myelotomy of about 3 cm was performed and a grayish, elastic and circumscribed tumor as well as a liquefied hematoma in the caudal part was observed. Both the tumor and the hematoma were removed almost totally. The patient's paraparesis improved slightly after surgery. The histological diagnosis was adenocarcinoma. The primary source was unknown, but multiple small metastatic tumors were found in the lung, liver and brain, etc. Hemorrhage from intramedullary spinal cord metastasis is extremely rare with only 6 reported cases in the recent literature. Rapid deterioration of symptoms caused by the hematoma may make the diagnosis more difficult. Indication of surgical treatment should be carefully determined because prognosis of intramedullary spinal cord metastasis is generally very poor.     

Primary holocord ganglioneuroblastoma: case report

The authors present a case of extensive primary intramedullary spinal CNS ganglioneuroblastoma (GNB) in a 23-year-old man. Central nervous system GNB is a poorly differentiated neuroepithelial tumor composed of neuroblasts and differentiated ganglion cells, and these lesions are extremely uncommon. Most previously reported primary intraaxial neuroblastic tumors were described in the brain. There has been only one other report of primary spinal cord CNS GNB published to date; the clinical course and prognosis for primary spinal cord tumors of this type are unknown. Similar tumor types demonstrate poor prognoses. This 23-year-old man presented after 9 months of progressive myelopathy. Admission MR imaging showed an intraaxial enhancing mass extending from C-3 to the conus medullaris, with a holocord appearance in several areas. Due to the tumor size, operative intervention was initially limited to biopsy sampling. Chemotherapy resulted in histological maturation, but initial tumor regression was temporary. The patient suffered progressive quadriparesis, and neuroimaging demonstrated slow enlargement of the tumor and an associated syrinx. Nineteen months after diagnosis, the tumor was excised to gross-total resection in a 2-stage operation. One year following resection, the patient had no radiographic recurrence and was functional in a wheelchair with minimal paresis in the upper extremities. This case represents the most extensive example of primary spinal intramedullary CNS GNB reported to date. Holocord tumors present a significant challenge to the neurosurgeon, and resection bears substantial risk of morbidity. In spinal cord CNS GNB, chemotherapy followed by complete resection may be the most effective means of tumor control.     

The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients

171 patients with intramedullary spinal cord tumors were operated on, of which 25 patients (15%), mostly children, developed symptomatic hydrocephalus. Twenty patients (12%) had malignant tumors, with 13 of the 20 cases (63%) complicated by increased intracranial pressure and ventriculomegaly. Of the remaining 151 patients with benign tumors (89%), only 12 (8%) developed symptomatic hydrocephalus. In an effort to understand the relationship between hydrocephalus and intramedullary spinal cord tumor, the authors analyze the level and histology of the neoplasm, as well as its association with spinal cysts. A review of the neurosurgical literature reveals that 34 similar cases of hydrocephalus associated with intramedullary spinal cord tumors have been reported to date. The authors note that the presence of hydrocephalus in patients with malignant intramedullary astrocytomas is associated with a shorter rate of survival than in those patients with high-grade lesions but without hydrocephalus, apparently due to rapid tumor progression. The ventriculomegaly seen with benign spinal cord gliomas has no statistically significant effect upon long-term prognosis.     

Adult supratentorial oligodendrogliomas. Surgical treatment: indications and techniques

Surgical resection is the first step in the treatment of adult supratentorial oligodendrogliomas (OLG). However, the role of resection on prognosis, the most appropriate time for surgery along the natural history of those tumors, and the best operative strategy remain debated. Survival curves after resection vary greatly among reported series, in particular as a result of a persisting confusion in identification and classification of cerebral OLG. Surgical or stereotactic biopsy is the first surgical procedure which enables confirmation of the diagnosis suggested on imaging, assessment of extension of tumor cell infiltration beyond abnormalities limit described an imaging, and currently available molecular biology studies. Biopsies may be the only surgical procedure in patients having a deep-seated tumor with minimal mass effect, or prior to a surgical resection or a "wait and watch" strategy. Surgical resection is indicated for the other patients. However, it has not been demonstrated that time for resection has an influence on survival, excepted in patients with rapidly growing tumors, with mass effect causing increased intracranial pressure. A wait and watch strategy is therefore warranted in patients with a tumor aspect suggestive of a grade A OLG; surgical resection may be indicated later. There is a current trend for maximal safe resection, preserving functional cerebral areas, since truly complete resection of the tumor including infiltration is exceptional. However, from the contradictory results reported to date, one cannot ascertain whether large or complete resection based on imaging is associated with significantly longer survival. Neuronavigation guidance, intraoperative imaging, and cortical stimulation techniques are helpful neurosurgical techniques enabling maximal safe resection with preservation of functional areas.     

Primary Spinal Neurocytoma Involving the Medulla Oblongata: Two Case Reports and a Literature Review

Central neurocytoma is a rare neuroectodermal tumor found in young adults. These tumors are generally located in the lateral or third ventricles. Extraventricular neurocytoma in the spinal cord is extremely rare. We report on two patients with primary spinal neurocytomas who presented with progressive numbness and weakness in the limbs. Both patients had intramedullary masses between the medulla and the upper thoracic levels. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all 20 reported cases were reviewed. In conclusion, neurocytoma should be included in the differential diagnosis of a spinal intramedullary tumor, and subtotal resection is acceptable for a relatively favorable prognosis if gross total removal is unachievable. The efficacy of adjuvant radiochemotherapy to control tumor recurrence is unknown.     

Intramedullary spinal teratoma presenting with urinary retention: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Intramedullary tumors affect a small but significant segment of patients with spinal cord tumors. Intramedullary teratomas are rare entities that are usually located in the sacrococcygeal region. Although some reports claim that diagnostic studies can exactly predict the nature of intramedullary tumors, this case report demonstrates the lack of preoperative diagnostic specificity. Therefore, the aim of surgery should be radical extirpation whenever possible. CLINICAL PRESENTATION: A case report of a thoracolumbar intramedullary teratoma is presented. This young male patient displayed urinary retention and motor deficits. Computed tomographic and magnetic resonance imaging examination revealed an intramedullary tumor; however, the exact nature of the tumor could not be determined preoperatively. The literature concerning these tumors is extensively reviewed. INTERVENTION: During surgery, a multicystic tumor was found at the base of the filum terminale, which was resected as far as possible. CONCLUSION: It is concluded that surgery should be the first choice for treatment. More aggressive teratomas are found in intramedullary locations, especially in children, emphasizing the importance of radical tumor resection. The use of adjuvant chemo- or radiotherapy should be explicitly substantiated for the nonbenign teratoma group.     

Holocord astrocytoma: a two-staged operative approach

Biopsy or subtotal resection followed by radiation for intramedullary astrocytomas of the spinal cord offer palliation but not cure. A treatment modality using an ultrasonic suction device has revolutionized the treatment of these tumors and has been extended to include the treatment of intramedullary tumors involving the entire length of the spinal cord (holocord tumors) with evident success. An alternative to this technique, a two-staged microsurgical approach, allows complete excision of the tumor and thus offers a chance for cure. Two patients have been treated in this manner, and more than 3 years after the operations, both patients were still improving neurologically without any evidence of tumor recurrence.     

Anterior approach to cervical intramedullary pilocytic astrocytoma. Case report

Although there has been considerable experience with anterior approaches to ventral intradural, extramedullary, and pial-based spinal lesions, there is no information in the literature regarding the safety and feasibility of the resection of an intramedullary tumor via an anterior approach. The authors report on the gross-total resection of an intramedullary cervical pilocytic astrocytoma via a C-7 corpectomy and anterior myelotomy. The surgery proceeded without complication, and postoperatively the patient maintained the preoperative deficit of mild unilateral hand weakness but had no sensory deficits. Follow-up MR imaging at 6 months showed gross-total macroscopic resection. Selected intramedullary tumors can be safely removed via an anterior approach. This approach avoids the typical sensory dysfunction associated with posterior midline myelotomy.     

Intramedullary spinal cord metastasis: two case reports and literature review

Intramedullary spinal cord metastasis was thought to be rare, but the advent of MR imaging and treatment for malignancy have revealed that it is more frequent than we previously thought. We report two cases of intramedullary spinal cord metastasis from lung and rectal colon carcinoma. Their symptoms were aggravated rapidly. We performed surgical treatment in both cases; one was grosstotal and the other was subtotal resection. After the operation, symptoms improved mildly in one patient and worsened in the other patient. Both died 3 months after surgery, because of respiratory disturbance due to brain stem compression and lung metastasis. In conclusion, MRI is highly useful for making a diagnosis of intramedullary spinal metastatic tumors. However, it is still difficult to treat such a patient by surgery alone. Further postoperative adjuvant therapy will be necessary to improve the prognosis of such patients.     

Dorsal intramedullary spinal epidermoid cysts: Report of two cases and review of literature

Intramedullary epidermoid cysts of the spinal cord are rare tumors, especially those not associated with spinal dysraphism. About 50 cases have been reported in the literature. Of these, only seven cases have had magnetic resonance imaging (MRI) studies. We report two cases of spinal intramedullary epidermoid cysts with MR imaging. Both were not associated with spina bifida. In one patient, the tumor was located at D4 vertebral level; while in the other, within the conus medullaris. The clinical features, MRI characteristics and surgical treatment of intramedullary epidermoid cyst are presented with relevant review of the literature.     

Postoperative prognostic factors in patients with spinal cord tumors]

This study is to analyse the prognostic factors of the patients with spinal cord tumors, consisted of intramedullary tumor (33 patients), multiple extramedullary tumor (15), solitary cauda equina tumor (22), and solitary extramedullary tumor (48). The significant prognosis of intramedullary tumors depends on the histopathological type, the tumor margin, and the extramedullary tumor extension. Multiple extramedullary tumors had a tendency to recur at the different spinal cord levels; poorer long-term clinical results than those with solitary spinal cord tumor. Solitary cauda equina tumor cases showed good clinical course except for the bladder disfunction. Prognostic factors for solitary extramedullary tumors were the preoperative neurological status, the duration of myelopathy prior to surgery, the age at surgery, the tumor size, tumor location along the spinal axis, and the site of tumor in relation to the spinal cord.     

Treatment of spinal cord tumors

Opinion statement  Tumors associated with the spinal cord can have devastating effects on patient function and quality of life. Most of these tumors are from metastatic disease, usually to the epidural space. Less frequently, the tumors are intrinsic to the spinal cord itself (ie, primary tumor). Regardless of the etiology, spinal cord tumors often present first with progressive local or radicular pain, or both. Other symptoms include weakness, sensory changes, or sphincter dysfunction. The timeliness of diagnosis of spinal cord tumors and promptness of treatment are important, as they directly affect outcome. Dexamethasone, a corticosteroid, is used as a temporizing measure to improve or stabilize neurologic function until definitive treatment. For nonambulatory patients with epidural metastatic tumors, surgery followed by radiation therapy maximizes neurologic function and modestly lengthens survival. However, palliative radiotherapy alone is recommended for those with neurologic deficits lasting longer than 48 hours, survival prognosis less than 3 months, inability to tolerate surgery, multiple areas of compression, or radiosensitive tumors. An ambulatory patient with a stable spine should be considered for radiation treatment only. The role of chemotherapy for epidural metastatic tumors is not well established. For intramedullary metastases, the role of surgery and chemo therapy remains controversial and radiation is the mainstay. For low-grade or benign primary spinal cord tumors, resective surgery is of benefit and can be curative. For high-grade tumors, the benefit of resection is less clear, and radiotherapy and/or chemotherapy may be helpful. The use of chemotherapy for primary spinal cord tumors has rarely been assessed. Agents reported in the literature for treatment of spinal cord gliomas include temozolomide, irinotecan, cisplatin, and carboplatin. A multidisciplinary approach is often required to maximize the therapeutic and functional outcome of patients with metastatic and primary spinal cord tumors.     

脊髓髓内肿瘤的显微手术治疗

目的:评估脊髓髓内肿瘤的手术疗效,了解影响预后的因素。方法:回顾分析42例显微手术治疗的髓内肿瘤患者的临床资料,颈髓28例,颈胸交界6例,胸髓5例,圆锥3例。结果:肿瘤全切30例,次全切8例,部分切除4例。无手术死亡。随访3~36个月,病情较术前改善者24例,稳定者13例,加重5例。术前神经功能障碍严重的患者,术后恢复差。3例星形细胞瘤患者术后8~16个月内死亡。结论:脊髓髓内肿瘤在显微镜下大多可手术全切或次全切,并保留正常的脊髓功能,改善或稳定病情。手术应在神经功能加重前进行。恶性胶质瘤的手术效果及预后仍不满意。     

Bilateral Posterolateral Sulcus Approach for the Removal of Spinal Intramedullary Metastatic Adenocarcinoma: A Technical Case Report

Spinal intramedullary metastasis is an extremely rare event that occurs in advanced cancer. The surgical indications for spinal intramedullary metastasis are highly limited because of surgical difficulty and poor prognosis. In this technical case report, we present a rare case of spinal intramedullary metastasis from the lung that recurred late after local radiation to the spinal cord. The patient progressively experienced relapsed buttock pain and developed gait and urination disorders late after treatment for lung cancer. Imaging examinations suggested the recurrence of spinal intramedullary metastasis in the conus medullaris. Systemic examinations revealed no apparent recurrence in other organs, including the primary lung lesions. Gross total resection of the tumor within the conus medullaris was safely performed using the unilateral posterolateral (PLS) approach and by addition of the contralateral PLS approach. To the best of our knowledge, this is the first case in which a spinal intramedullary metastatic tumor was successfully removed using a bilateral PLS approach.     

Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: Case report and review of the literature

Context

Hemangioblastomas of the spinal cord are uncommon vascular tumors. Patients commonly present with subtle neurologic findings that are thought to represent growth of the lesion over time. Hemorrhage of an intramedullary hemangioblastoma presenting as acute neurologic deficit is an extremely rare occurrence. Although the cervical spine is the most common location for hemangioblastoma of the spinal cord, there have been no previously published cases in the literature of intramedullary hemorrhage from such a lesion.

Findings

A 22-year-old woman with a previously undiagnosed spinal cord hemangioblastoma presented with sudden-onset dense quadriparesis due to intramedullary hemorrhage in the cervical spinal cord. The patient did not have any clinical findings of von-Hippel Lindau disease. Laminoplasty from C5 to T2 and posterior midline myelotomy for resection of the intramedullary tumor with hematoma evacuation were completed without complication.

Conclusion

Intramedullary hemangioblastoma of the spinal cord is uncommon, and hemorrhage from a cervical spinal cord lesion has not previously been reported. Symptoms from these usually indolent lesions are commonly associated with tumor growth, edema, or associated syrinx, whereas devastating acute neurologic deficit from hemorrhage is exceedingly rare. Microsurgical resection should be done in cases of symptomatic lesions and considered in isolated symptomatic lesions without the known diagnosis of von Hippel-Lindau disease.     

Unilateral,multilevel, interlaminar fenestration in the removal of a multisegment cervical intramedullary ependymoma

Background contextLaminectomy is the traditional approach to intramedullary ependymomas and may lead to spinal instability and spinal deformities. Hemilaminectomy and laminoplasty have been developed to preserve the spinal stability and have been found to be effective. Unilateral, multilevel, interlaminar fenestration is another approach that may have more advantages in preserving the spinal stability; however, it has rarely been used by now.PurposeUnilateral, multilevel, interlaminar fenestration was often used in the surgical treatment of degenerative lumbar stenosis; however, it was rarely used for tumors. The aim of this study was to discuss the characteristics and advantages of its use for a multisegment intramedullary ependymoma.Study designCase report and literature review.MethodsThe 22-year-old man suffered from muscular atrophy of the left hand and the right hand for a period of 1.5 years and 3 months, respectively, and the cervical magnetic resonance imaging revealed an intramedullary mass of C4–C7. A right, unilateral, multilevel, interlaminar fenestration of the C3–C7 was performed with the help of high-speed air drills, and a midline myelotomy was made under microscope. The tumor was removed totally using piecemeal resection.ResultsThe magnetic resonance imaging, obtained 1 year after the operation, revealed that there were no residual mass lesion and no spinal instability, and the patient acquired an excellent functional outcome. So, this technique proved to be safe and easy in this case.ConclusionsUnilateral, multilevel, interlaminar fenestration allows good access to a long intraspinal segment, protects the structures essential to spinal stability as much as possible, preserves the spinal stability, and results in no additional injury to the spinal cord. It may be applicable to many other kinds of intraspinal tumors.     

Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases

Two cases of primary extraosseous intradural spinal Ewing’s sarcoma are reported with a review of the current literature. This rare neoplasm shares features with cerebral primitive neuroectodermal tumors, complicating a correct diagnosis. Gross total resection seems to be the main treatment, although adjuvant therapies could improve the prognosis. In case 1, a 56-year-old man presented with cauda equina syndrome. MRI showed an intradural tumor from L4 to S2. An emergency laminectomy was performed with gross total resection of a hemorrhagic tumor, followed by adjuvant treatment. In the second case, a 25-year-old female developed leg and lumbar pain. MRI study identified a homogeneously enhancing intradural mass at the L2-L3 level. A laminoplasty was performed, followed by tumor resection; no adjuvant treatment was administered afterwards. Immunohistochemical workup confirmed the diagnosis of Ewing’s sarcoma in both cases.     

Intramedullary spinal cord tumors

Opinion statement The three most common types of intramedullary spinal cord tumors are low-grade astrocytomas, ependymomas, and high-grade astrocytomas. Surgical extirpation is the necessary and sufficient primary treatment for most intramedullary spinal cord tumors. Radiation therapy may also have a role in the management of persistent, recurrent, or progressive low-grade astrocytomas and ependymomas. The current treatment of spinal cord high-grade astrocytomas, which includes surgical debulking, radiation therapy, and possibly chemotherapy, is clearly inadequate. Chemotherapy may have a potential role for certain progressive spinal cord tumors, but the role is undefined at present. Recent reports have described the use of stereotactic radiosurgery for extramedullary spinal tumors, and stereotactic radiosurgery may someday be useful in the management of intramedullary spinal cord tumors. Rehabilitation programs are an important component of the multidisciplinary care of patients with spinal cord tumors. Finally, more work, especially the inclusion of adults and children with intramedullary spinal cord tumors into prospective clinical trials, is needed to improve the therapy of intramedullary spinal cord tumors and rehabilitation after diagnosis of a spinal cord tumor.