Cases reported "Spinal Cord Neoplasms"

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1/35. Combined posterior and posterolateral one-stage removal of a giant cervical dumbbell schwannoma.

    OBJECTIVE: Huge dumbbell schwannomas in the cervical region are not a rare clinical entity in neurosurgical practice. Despite the benign nature, the adhesions between tumor capsule and spinal cord, vertebral artery, cervical plexus, and carotid sheath can complicate surgery, leading to a fatal outcome. We performed one-stage combined surgery through the posterior midline and an unusual posterolateral cervical route in the same position and describe its advantages. METHOD: We removed the intraspinal component totally through a posterior midline approach and freed spinal cord and vertebral artery. After removal of the posterolateral extraspinal component using the same incision, a posterolateral cervical approach along the posterior margin of sternocleidomastoid muscle was performed, and the anterolateral extraspinal tumor was excised totally through the dissection plane between anterior and middle scalene muscles. RESULTS AND CONCLUSIONS: The operative time was less than 3 h, and the patient was discharged on the 3rd postoperative day without any neurological sequel. The combination of posterior midline and posterolateral approach in large dumbbell schwannomas is easier to perform and a more safe technique than the combination with anterolateral accesses in the cervical region and also less invasive than the transuncodiscal technique in selected cases. This approach reduces the risk of neurovascular injury, since it does not require retraction and repositioning of the patient and, therefore, facilitates total excision and reduces the operative time.
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2/35. 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.
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3/35. Sinus histiocytosis with massive lymphadenopathy and spinal epidural involvement: a case report and review of the literature.

    A case of "sinus histiocytosis with massive lymphoadenopathy" (SHML) in which spinal epidural involvement caused paraplegia is described. The literature pertaining to this newly recognized entity is reviewed; this review indicates that the process is not necessarily restricted to the lymph nodes and may be progressive in nature. The case we report is the first with histologically proved bone involvement.
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4/35. spinal cord pilocytic astrocytoma with cranial meningeal metastases.

    Dissemination of tumour cells along the cerebrospinal fluid (CSF) pathway has been reported mostly in medulloblastomas, germ cell tumours or high grade gliomas. Juvenile pilocytic astrocytoma (JPA) is usually a benign astrocytoma. However, drop metastases of indolent nature from intracranial tumours to the spinal cord are documented. All of the previously reported cases represent metastases of cerebellar or hypothalamic tumours spreading to the spinal cord. We document in this paper the first report of a spinal cord pilocytic astrocytoma spreading via the CSF to the cerebral meninges. A 9 year old girl had a JPA of C5 to C7 subtotally resected. Two and a half years later she presented with hydrocephalus with radiologically meningeal enhancement. The meninges were biopsied which showed metastatic JPA. The girl was relatively well 4 years after initial surgery with residual tumour. spinal cord JPA can rarely metastasize to the cranial meninges. Similar to intracranial tumours which spread to the spinal cord, such metastatic lesions are indolent.
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5/35. Primary rhabdomyosarcoma of the central nervous system.

    A case of primary rhabdomyosarcoma in the brain stem is described in an 8 year old girl. The clinical data showed a right side hemiplegia then a total paralysis of the left sixth cranial nerve and a paraplegia which became lethal in 3 months. The necrospy revealed a tumoral nodule in the left medulla oblongata and pons with diffuse subarachnoidal extension from the cranial nerves to the cauda equine roots. Histologically the tumor appeared to be polymorph with numerous rhabdomyoblasts which had a clear cross striation and which were sometimes less differentiated without any neuronal or glial elements. Perivascular tumoral cells and blood vessels were closely linked, the Virchow-Robin spaces were clearly involved. The electron microscopic study confirmed that the less differentiated cells were of a rhabdomyoblastic nature. A review of the litterature indicates that these malignant neoplasias are highly exceptional, and can be classified within the group of primary tumors of the neuraxis with muscular elements. The histogenetic origin of these tumors appears to be the ectomesenchyme of neural crests.
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6/35. Surgical management of spinal cord hemangioblastomas in patients with von hippel-lindau disease.

    OBJECT: Von Hippel-Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients. methods: Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20-58 years) at the National Institutes of health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered. CONCLUSIONS: Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.
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7/35. Primary intramedullary spinal cord germinoma: case report.

    We describe an 18-year-old male with an intramedullary spinal cord germinoma in whom the diagnosis was made two years after onset of a progressive myelopathy. spinal cord germinomas are rare, most having been described in young Japanese adults. They often respond well to radiotherapy. A unique feature of this case was the prolonged time interval between onset of the patient's symptoms and ability to visualize the mass radiographically. A further interesting finding was the infiltrative nature of the tumor surrounding residual spinal cord neurons.
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8/35. Spinal intramedullary teratoma with exophytic components: report of two cases and review of the literature.

    True intramedullary teratoma is an extremely rare tumor, with only seven cases reported in the literature. The authors present two personal cases of spinal intramedullary cystic teratoma of the conus medullaris with exophytic growth and some unusual aspects. Their cases are unusual not only because they were diagnosed by MRI but also because the monitored microsurgical removal of the tumor was apparently total, with excellent results, in clinical and neuroradiologic terms. We recommend early radical surgery whenever possible, when the patient's neurological status is still good. To prevent traumatizing adjacent spinal cord for cases in which the teratoma tightly adheres to the functional neurological tissue of the spinal cord with no cleavage plane, we do not recommend an aggressive surgical attempt. Because of the mostly benign nature of this disease, the symptomatic recurrence of such incompletely resected mature teratomas is slow and may eventually require a second surgical procedure. The relevant literature is also reviewed.
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9/35. Cellular schwannoma: a rare spinal benign nerve-sheath tumor with a pseudosarcomatous appearance: case report.

    We report a case of cellular schwannoma, a rare benign nerve-sheath tumor in a 27 year-old woman. It was presented as a voluminous lesion in the paraspinal region that caused lumbar vertebral body destruction. These features, in association to the microscopic aspects of a hypercellular, pleomorphic neoplasm may lead to a false impression of a malignant tumor. Therefore, it is important to have an accurate examination to confirm the benign nature of this tumor thus avoiding unnecessary therapy.
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10/35. Pilomyxoid astrocytoma of the spinal cord: report of three cases.

    OBJECTIVE AND IMPORTANCE: Pilomyxoid astrocytoma (PMA) is a recently described, rare, circumscribed glioma similar to pilocytic astrocytoma. Despite its circumscribed nature, PMA implies a worse overall outcome than typical pilocytic astrocytoma. All PMAs reported to date involved the hypothalamic/chiasmatic region. Since these original reports, we have encountered three tumors in the spinal cord with histological features identical to those of PMA. CLINICAL PRESENTATION: We report three male pediatric patients. The first patient presented at age 6 with back pain and constipation. The second patient presented at age 8 with back pain and a bout of urinary incontinence. The third patient, a neonate, presented with respiratory failure and hypotonia. INTERVENTION: The first patient's cervical and thoracic neoplasm was treated with subtotal resection and multiple laminectomies and spinal fusion for recurrence. The second patient underwent gross total resection of his thoracic neoplasm. The neonate underwent laminectomy and biopsy of his holocord tumor. CONCLUSION: PMAs typically occur in the hypothalamic region and within the first 4 years of life. Tumors with identical histological features are being recognized outside this typical clinical setting. The cases presented in this report suggest that PMA can occur at a later age and can involve the spinal cord. We believe that increased recognition of these circumscribed gliomas will help to elucidate their nature and lead to better management decisions.
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